Aims. To determine whether side-bending films in scoliosis are assessed for adequacy in clinical practice; and to introduce a novel method for doing so. Methods. Six surgeons and eight radiographers were invited to participate in four online surveys. The generic survey comprised erect and left and right bending radiographs of eight individuals with scoliosis, with an average age of 14.6 years. Respondents were asked to indicate whether each bending film was optimal (adequate) or suboptimal. In the first survey, they were also asked if they currently assessed the adequacy of bending films. A similar second survey was sent out two weeks later, using the same eight cases but in a different order. In the third survey, a guide for assessing bending film adequacy was attached along with the radiographs to introduce the novel T1-45B method, in which the upper endplate of T1 must tilt ≥ 45° from baseline for the study to be considered optimal. A fourth and final survey was subsequently conducted for confirmation. Results. Overall, 12 (86%) of 14 respondents did not use any criteria to assess the bending film adequacy; the remaining two each described a different invalidated method. In total, 12 (86%) of the respondents felt T1-45B was easy to learn and apply. There was fair to substantial intra-rater reliability (k = 0.25 to 0.88) which improved to fair to almost perfect (k = 0.38 to 0.88) post-introduction of the guide. Inter-rater reliability varied considerably among the rater groups but similarly increased following introduction of the guide (k. S1. = 0.19 to 0.34, k. S2. = 0.33 to 0.43 vs k. S3. = 0.49 to 0.5, k. S4. = 0.35 to 0.43). Conclusion. Many surgeons and radiographers do not assess spinal bending films for adequacy. We propose that the change in the plane of the upper endplate of T1 on side-bending can be used in this evaluation. In the T1-45B method, a change of ≥ 45° on side bending qualifies as an adequate bend effort. Cite this article: Bone Jt Open 2023;4(9):689–695

Aims. Vertebral body tethering (VBT) is a non-fusion technique to correct scoliosis. It allows correction of scoliosis through growth modulation (GM) by tethering the convex side to allow concave unrestricted growth similar to the hemiepiphysiodesis concept. The other modality is anterior scoliosis correction (ASC) where the tether is able to perform most of the correction immediately where limited growth is expected. Methods. We conducted a retrospective analysis of clinical and radiological data of 20 patients aged between 9 and 17 years old, (with a 19 female: 1 male ratio) between January 2014 to December 2016 with a mean five-year follow-up (4 to 7). Results. There were ten patients in each group with a total of 23 curves operated on. VBT-GM mean age was 12.5 years (9 to 14) with a mean Risser classification of 0.63 (0 to 2) and VBT-ASC was 14.9 years (13 to 17) with a mean Risser classification of 3.66 (3 to 5). Mean preoperative VBT-GM Cobb was 47.4° (40° to 58°) with a Fulcrum unbend of 17.4 (1° to 41°), compared to VBT-ASC 56.5° (40° to 79°) with 30.6 (2° to 69°)unbend. Postoperative VBT-GM was 20.3° and VBT-ASC Cobb angle was 11.2°. The early postoperative correction rate was 54.3% versus 81% whereas Fulcrum Bending Correction Index (FBCI) was 93.1% vs 146.6%. The last Cobb angle on radiograph at mean five years’ follow-up was 19.4° (VBT-GM) and 16.5° (VBT-ASC). Patients with open triradiate cartilage (TRC) had three over-corrections. Overall, 5% of patients required fusion. This one patient alone had a over-correction, a second-stage tether release, and final conversion to fusion. Conclusion. We show a high success rate (95%) in helping children avoid fusion at five years post-surgery. VBT is a safe technique for correction of scoliosis in the skeletally immature patient. This is the first report at five years that shows two methods of VBT can be employed depending on the skeletal maturity of the patient: GM and ASC. Cite this article: Bone Jt Open 2022;3(2):123–129

Aims. Magnetically controlled growing rods (MCGR) have been gaining popularity in the management of early-onset scoliosis (EOS) over the past decade. We present our experience with the first 44 MCGR consecutive cases treated at our institution. Methods. This is a retrospective review of consecutive cases of MCGR performed in our institution between 2012 and 2018. This cohort consisted of 44 children (25 females and 19 males), with a mean age of 7.9 years (3.7 to 13.6). There were 41 primary cases and three revisions from other rod systems. The majority (38 children) had dual rods. The group represents a mixed aetiology including idiopathic (20), neuromuscular (13), syndromic (9), and congenital (2). The mean follow-up was 4.1 years, with a minimum of two years. Nine children graduated to definitive fusion. We evaluated radiological parameters of deformity correction (Cobb angle), and spinal growth (T1-T12 and T1-S1 heights), as well as complications during the course of treatment. Results. The mean Cobb angles pre-operatively, postoperatively, and at last follow-up were 70° (53 to 103), 35° (15 to 71) and 39° (15 to 65) respectively (p < 0.001). Further, there was a mean of 14° (-6 to 27) of additional Cobb angle correction upon graduation from MCGR to definitive fusion. Both T1-T12 and T1-S1 showed significant increase in heights of 27 mm and 45 mm respectively at last follow-up (p < 0.001). Ten children (23%) developed 18 complications requiring 21 unplanned operations. Independent risk factors for developing a complication were single rod constructs and previous revision surgery. Conclusion. MCGR has the benefit of avoiding multiple surgeries, and is an effective tool in treatment of early-onset scoliosis. It also maintains the flexibility of the spine, allowing further correction at the time of definitive fusion. Cite this article: Bone Joint Open 2020;1-7:405–414

Abstract. MAGnetic Expansion Control (MAGEC) rods are used in the surgical treatment of children with early onset scoliosis. The magnetically controlled lengthening mechanism enables rod distractions without the need for repeated invasive surgery. The CE certification of these devices was suspended in March 2021 due, primarily, to performance evidence gaps in the documents provided by the manufacturer to regulators and notified bodies. MAGEC rods are therefore not permitted for use in countries requiring CE marking. This was a survey of 18 MAGEC rod surgeons in the UK about their perception of the impact of the CE suspension on the clinical management of their patients. Unsurprisingly, virtually all perceived a negative impact, reflecting the complexity of this patient group. Reassuringly, these surgeons are highly experienced in alternative treatment methods. Cite this article: Bone Jt Open 2022;3(2):155–157

Despite the myriad new spinal instrumentation systems, scoliosis can rarely be fully corrected, especially when the curves are stiff. A novel superelastic nickel-titanium (nitinol) rod that maximises the ability to slowly correct spinal deformities by utilising the viscoelastic properties of the spine has been developed. This parallel, double-blinded, randomised controlled trial compared the safety and efficacy of these new rods to conventional titanium rods in 23 patients with adolescent idiopathic scoliosis. The superelastic nitinol rods were found to be safe, could gradually correct scoliosis curves, and ultimately resulted in better coronal and sagittal alignments compared to traditional rods. Despite the myriad new spinal instrumentation systems, scoliosis can rarely be fully corrected, especially when the curves are stiff. A novel superelastic nickel-titanium (nitinol) rod that maximises the ability to slowly correct spinal deformities by utilising the viscoelastic properties of the spine has been developed. This parallel, double-blinded, randomised controlled trial compared the safety and efficacy of these new rods to conventional titanium rods in 23 patients with adolescent idiopathic scoliosis. The superelastic nitinol rods were found to be safe, could gradually correct scoliosis curves, and ultimately resulted in better coronal and sagittal alignments compared to traditional rods

Objective: To study on the therapeutic mechanism of an innovated instrumentation--Plate-Rod System for scoliosis (PRSS) and its effectiveness for the surgical management of early on-set scoliosis (EOS). Method: Between June 2000 and July 2008, 23 patients with progressive EOS who underwent one stage PRSS procedure without bony fusion.and had been followed-up for more than 2 years were evaluated prospectively. The mean age at the time of surgery was 7.98 years The experimental studies including: X-ray analysis; photo-elastic test and type ~ collagen were studied to express the therapeutic mechanism. Results: The mean follow up period was 2.8 ±1.4 years, more than 5 years in 5 cases. The mean scoliosis improved from 80.7° to 30.5° after surgery with a corrective rate of 62.2% and in latest follow.-up was 34.7° The length of the growth of the instrumented spine was average 13.3mm. No severe complications in our series. When PRSS is placed in place, compressive stress was found to exert on the convex side, while tensile stress on the concave side of the curvature which were reflected by the changes on the color band in the photo-elastic test and by the changes in width of the disc spaces, and more type X collagen expressed on convex side than concave side, it suggest that compressive stress leads to increase earlier cartilage degeneration of end plate in convex side correlating with the decreased growth of the end plate of this side, and resulting in maximum spinal realignment. Conclusion: The PRSS which dispenses with spinal fusion and allows extension along with the children’s growth, is able to provide and maintain desirable correction of scoliosis in the later growing year due to its modulating efficiency in normalizing the spinal growth, This new device is an effective instrumentation for correcting scoliosis, especially for EOS

Scoliosis requires three dimensional correction at a global level (curve correction) and at a local one (apical axial derotation) as well as sagittal balance management. Except for in situ contouring, previously reported surgical techniques for scoliosis correction hardly deal with all these issues. The aim of the current study was to evaluate long term clinical and radiological outcomes after in situ contouring in 85 patients with severe scoliosis (Cobb= 40 to 110°). Age influence (adults versus adolescents) and surgical approaches (anterior release and posterior correction and fusion versus posterior correction and fusion only) were also assessed. The results of the study show that the in situ contouring is comparable to other surgical techniques in terms of surgery duration and blood loss. Anterior release proved useful in severe scoliosis correction. No difference in peroperative complications was found between age groups nor between approach groups. However, adolescents recover faster than adults. No difference of revision rates in double approach versus posterior approach populations was found. No statistically significant differences were found between the adolescent and adult populations. The mean overall frontal correction reached 68%. The mean loss of correction amounted 5%. No significant evolution was found in sagittal curvatures, emphasizing the difficulties in restoring physiological curvatures in patients with severe scoliosis. Our results suggest the in situ contouring technique is fully appropriate for severe scoliosis correction, regardless of the patient’s age and approach. Besides it will not result in higher morbidity for one specific population and warrants similar outcome when properly applied

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

Aim. To investigate anterior instrumented corrective fusion for thoracolumbar or lumbar scoliosis. Methods. A retrospective review of medical records and radiographs of 38 consecutively managed patients who underwent anterior spine surgery for thoracolumbar curves by a single surgeon between 2001 and 2011. The cohort consisted of 28 female and 10 male patients with idiopathic scoliosis as the commonest aetiology. Data collated and analysed included patient demographics, surgical factors, post-operative management and complications. In addition, radiographic analysis was performed on pre-operative and follow-up x-rays. Results. Thoracolumbar/lumbar curves were corrected from 70 to 27 degrees. The thoracic compensatory curve spontaneously corrected from 34 to 19 degrees. Sagittal imbalance of greater than 4 centimeters was found in 40 percent of patients preoperatively and in 16 percent post operatively (85 percent negative sagittal imbalance, 15 percent positive sagittal imbalance). Rotation according to the Nash-Moe method corrected by 1.13 of a grade. Average operative time was 194 minutes and estimated blood loss was 450 ml. The diaphragm was taken down in 36 of the 38 patients but no post-op ventilation was required. The average high care stay was 1.2 days. Average follow-up was 18 months. Good maintenance of correction was shown at most recent follow-up, with the mean thoracolumbar/lumbar curve measuring 29 degrees, and the mean compensatory thoracic curve measuring 21 degrees. There were no significant neurological or respiratory complications. Conclusion. Anterior corrective fusion for thoracolumbar and lumbar scoliosis is effective in both deformity correction and maintenance thereof. Spontaneous correction of the thoracic curve can be expected and thus limit the fusion to the lumbar curve. Despite the concerns of taking down the diaphragm, there is minimal morbidity. NO DISCLOSURES

Purpose of study. The aim is to assess the use of non-fusion instrumentation “growth rods” in early onset scoliosis (EOS). Methods. A retrospective review of 12 consecutive patients who had undergone a growth rod procedure for EOS was performed. Six patients had neuromuscular scoliosis, 5 had juvenile idiopathic scoliosis while one had a congenital aetiology. Growth constructs were predominately constructed from modular commercially available sets using hooks, screws and connection blocks. One VEPTR was used in a severe kyphoscoliosis. Patients returned to theatre at 6 monthly intervals for a lengthening procedure. Patients were assessed with regards to age at presentation, age at surgery, indications for surgery, initial Cobb angle, post- operative Cobb angle, number of lengthening's done, instrumentation used, amount of spine growth achieved and complications. Results. The average age at presentation was 3 yrs 8 months (birth – 7 years 5 months). The average Cobb angle was 55 (38–90). Age at index surgery ranged from 2yrs 9 months to 8 years 2 months. The Cobb angle after the first procedure averaged 37 (range 20–90). The average lengthening over 51 lengthening procedures was 8 mm. Four patients have reached the end of the process and under gone a definitive fusion with pedicle screws and growth rods. Their final Cobb angle averaged 32 (26–48). Definitive surgery was performed earlier than planned in one patient due to repetitive rod breakage. Lengthening was abandoned in one patient whose implants became septic and required removal. Two patients required revision for superior construct failure. Conclusion. The growth rod procedure allows spinal deformity correction and control as well as on-going growth in trunk height. It is a labour intensive process with a significant incidence of complications. There is however very little choice in these patients due to concerns of fusion restricting pulmonary development. NO DISCLOSURES

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.

Introduction. The correction of paediatric scoliosis is a surgical procedure in which substantial bleeding occurs, and which historically has been associated with high levels of blood transfusion. Transfusions are associated with risks of infection, allergic reactions, volume overload and immunosuppression. The purpose of our study was to evaluate the efficacy of a care pathway implemented at St Thomas' hospital over ten years with a focus on minimising the need for blood transfusions. A Spinal Surgery Care Pathway was developed including: nurse-led clinics facilitating pre-operative haemoglobin optimisation; intra operative cell-salvage, the use of tranexamic acid, and a transfusion criteria awareness programme. Methods. We reviewed the records of all patients 18 years of age or younger undergoing spinal corrective surgery between 2000 and 2010. Collating data from the surgical records and haematology laboratory, we identified the proportion of patients requiring transfusion and the respective volume of blood or blood products transfused. Results. 534 spinal procedures were undertaken on patients over the decade of interest. Over this time, the proportion of patients requiring transfusion reduced from 83.3% in 2000, to 6.4% in 2010 (p < 0.001); a consistent year on year reduction was witnessed between 2005 and 2010. In those requiring transfusion, the volume of packed red cells transfused fell from a mean of 7.7 units in 2000, to 1.5 units in 2010 (p < 0.001). These results were despite an increase in posterior fusions, associated with higher bleeding, from 50.0% to 79.4% over the same time period. Conclusion. Over ten years, we have demonstrated that the implementation of a multifaceted, multidisciplinary pathway together with challenging cultural attitudes to blood transfusion, can dramatically and sustainably reduce the need for blood transfusion and the risks associated therewith. These data lends weight to the adoption of such a care pathway in paediatric scoliosis surgery

Introduction. Congenital scoliosis is a prevalent congenital spinal deformity, more frequently encountered than congenital lordosis or kyphosis. The prevailing belief is that most instances of congenital scoliosis are not hereditary but rather stem from issues in fetal spine development occurring between the 5th and 8th weeks of pregnancy. However, it has been linked to several genes in current literature. Our goal was to explore potential pathways through an exhaustive bioinformatics analysis of genes related to congenital scoliosis. Method. The literature from the 1970s to February 2024 was surveyed for genes associated with CS, and 63 genes were found to be associated with AIS out of 1743 results. These genes were analyzed using DAVID Bioinformatics. Result. Our pathway analysis has unveiled several significant associations with congenital scoliosis. Notably, “Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate” (P-Value:8.8E-3, Fold Enrichment: 20.6), “Central carbon metabolism in cancer” (P-Value:1.3E-3, Fold Enrichment: 10.3), and “Lysine degradation” (P-Value: 9.0E-3, Fold Enrichment: 9.1) emerge as statistically significant pathways. Additionally, “Endocrine resistance” (P-Value:4.4E-3, Fold Enrichment:7.4) and”EGFR tyrosine kinase inhibitor resistance” (P-Value: 1.7E-2, Fold Enrichment:7.3) pathways are noteworthy. These findings suggest a potential involvement of these pathways in the biological processes underlying congenital scoliosis. Furthermore, “Signaling pathways regulating pluripotency of stem cells” (P-Value:4.0E-4, Fold Enrichment:7.1), “Notch signaling pathway” (P-Value:6.7E-2, Fold Enrichment: 7.0), and “TGF-beta signaling pathway” (P-Value:6.2E-3, Fold Enrichment: 6.7) exhibit a less pronounced yet intriguing association that may warrant further investigation. Conclusion. In conclusion, our comprehensive analysis of the genetic etiology of congenital scoliosis has revealed significant associations with various pathways, shedding light on potential underlying biological mechanisms. While further research is needed to fully understand these associations and their implications, our findings provide a valuable starting point for future investigations into the management and treatment of congenital scoliosis

Prior to the introduction of steroid management in Duchenne Muscular Dystrophy (DMD), the prevalence of scoliosis approached 100%, concomitant with progressive decreases in pulmonary function. As such, early scoliosis correction (at 20-25°) was advocated, prior to substantial pulmonary function decline. With improved pulmonary function and delayed curve progression with steroid treatment, the role of early surgery has been questioned. The purpose of this study was to compare the post-operative outcomes of early versus late scoliosis correction in DMD. We hypothesize that performing later surgery with larger curves would not lead to worse post-operative complications. Retrospective cohort study. Patients with DMD who underwent posterior scoliosis correction, had pre-operative pulmonary function testing, and at least 1-year post-operative follow-up, were included; divided into 2 Groups by pre-operative curve angle – 1: ≤45°, 2: >45°. Primary outcome was post-operative complications by Clavien-Dindo (CD) grading. Secondary outcomes included: age at surgery, forced vital capacity (FVC), steroid utilization, fractional shortening (FS) by echocardiogram, surgery duration, blood transfusion requirements, ICU length of stay (LOS), days intubated post-operatively, hospital LOS, infection, curve correction. Two-tailed t-test and chi-square testing were used for analysis of patient factors and CD complication grade, respectively. 31 patients met the inclusion criteria, with a mean total follow-up of 8.3±3.2 years. Steroid treatment (prednisone, deflazacort) was utilized for 21 (67.7%) patients, for a mean duration of 8.2±4.0 years. Groups were comparable for steroid use, FVC, echo FS, and age at surgery (p>0.05). Primary curve angle was 31.7±10.4° and 58.3±11.1° for Groups 1 and 2, respectively (p 0.05). Surgery duration, ICU LOS, days intubated, hospital LOS, were also not different between Groups. For the entire cohort, however, the overall complication rate was higher for patients with steroid treatment [61.9% vs 10%, respectively (p=0.008)], the majority being CDII. Neither FVC nor echo FS were different between Groups at final follow-up (p=0.6; p=0.4, respectively). Post-operative complication rates were not different for early and late scoliosis correction in DMD. In general, however, patients undergoing steroid treatment were at higher risk of blood transfusion and deep infection. Delaying scoliosis correction in DMD while PF is favourable is reasonable, but patients with prior steroid treatment should be counseled regarding the higher risk of complications

Abstract. Aims. Vertebral body tethering (VBT) is a non-fusion technique to correct scoliosis allowing correction of scoliosis through growth modulation (GM) by tethering the convex side to allow concave unrestricted growth similar to the hemiepiphysiodesis concept. The other modality is anterior scoliosis correction (ASC) where the tether is able to perform most of the correction immediately where limited growth is expected. Methods. A retrospective analysis of 20 patients (M:F=19:1 – 9–17 years) between January 2014 to December 2016 with a mean five-year follow-up (4 to 7). Results. There were ten patients in each group with a total of 23 curves operated upon. VBT-GM mean age −12.5 years (9 to 14), mean Risser of 0.63 (0 to 2) and VBT-ASC was 14.9 years (13 to 17) and mean Risser of 3.66 (3 to 5). Mean preoperative VBT-GM Cobb was 47.4° (40°–58°) compared to VBT-ASC 56.5° (40°–79°). Postoperative VBT-GM Cobb was 20.3° and VBT-ASC was 11.2°. The early postoperative correction rate was 54.3% versus 81% whereas Fulcrum Bending Correction Index (FBCI) was 93.1% vs 146.6%. Latest Cobb angle at mean five years' follow-up was 19.4° (VBT-GM) and 16.5° (VBT-ASC). Overall, 5% of patients required fusion. Conclusion. We show a high success rate (95%) in helping children avoid fusion at five years post-surgery. VBT is a safe technique for scoliosis correction in the skeletally immature patient. This is the first report at five years showing two possible options of VBT depending on the skeletal maturity of the patient: GM and ASC

Clinically significant proximal junctional kyphosis (PJK) occurs in 20% of children treated with posterior distraction-based growth friendly surgery. In an effort to identify modifiable risk factors, it has been theorized biomechanically that low radius of curvature (ROC) implants (i.e., more curved rods) may increase post-operative thoracic kyphosis, and thus may pose a higher risk of developing PJK. We sought to test the hypothesis that EOS patients treated with low ROC (more curved rods) distraction-based treatment will have a greater risk of developing PJK as compared to those treated with high ROC (straighter) implants. This is a retrospective review of prospectively collected data obtained from a multi-centre EOS database on children treated with rib-based distraction with minimum 2-year follow-up. Variables of interest included: implant ROC at index (220 mm or 500 mm), patient age, pre-operative scoliosis, pre-operative kyphosis, and scoliosis etiology. In the literature, PJK has been defined as clinically significant if revision surgery with superior extension of the upper instrumented vertebrae was performed. In 148 scoliosis patients, there was a higher risk of clinically significant PJK with low ROC (more curved) rods (OR: 2.6 (95%CI 1.09-5.99), χ2 (1, n=148) = 4.8, p = 0.03). Patients had a mean pre-operative age of 5.3 years (4.6y 220 mm vs 6.2y 500 mm, p = 0.002). A logistic regression model was created with age as a confounding variable, but it was determined to be not significant (p = 0.6). Scoliosis etiologies included 52 neuromuscular, 52 congenital, 27 idiopathic, 17 syndromic with no significant differences in PJK risk between etiologies (p = 0.07). Overall, patients had pre-op scoliosis of 69° (67° 220mm vs 72° 500mm, p = 0.2), and kyphosis of 48° (45° 220mm vs 51° 500mm, p = 0.1). The change in thoracic kyphosis pre-operatively to final follow up (mean 4.0 ± 0.2 years) was higher in patients treated with 220 mm implants compared to 500 mm implants (220 mm: 7.5 ± 2.6° vs 500 mm: −4.0 ± 3.0°, p = 0.004). Use of low ROC (more curved) posterior distraction implants is associated with a significantly greater increase in thoracic kyphosis which likely led to a higher risk of developing clinically-significant PJK in EOS patients

Children undergoing posterior spinal fusion (PSF) for neuromuscular and syndromic scoliosis were admitted to the paediatric intensive care (PIC) until about 6 years ago, at which time we created a new unit, a hospital floor-based spinal high-dependency unit-plus (SHDU-plus), in response to frequent bed-shortage cancellations. This study compares postoperative management on PIC with HDU-plus for these non-hospital floor suitable children with syndromic and neuromuscular scoliosis undergoing PSF. Retrospective review of 100 consecutive children with syndromic and neuromuscular scoliosis undergoing PSF between June 2016 and January 2022. Inclusion criteria were: 1) diagnosis of syndromic or neuromuscular scoliosis, 2) underwent PSF, 3) not suitable for immediate postoperative hospital floor-based care. Exclusion criteria were children with significant cardio-respiratory co-morbidity requiring PIC postoperatively. 55 patients were managed postoperatively on PIC and 45 on SHDU-plus. No significant difference between groups was found with respect to age, weight, ASA grade, preoperative Cobb angles, operative duration, number of levels fused and estimated blood loss. 4 patients in the PIC group and 1 in the SHDU-plus group were readmitted back to PIC or HDU following step-down to the hospital floor. Average length of stay was 2 days on PIC and 1 day on SHDU-plus. Average total length of hospital stay was 16.5 days in the PIC group and 10.5 days in the HDU-plus group. 19 (35%) patients developed complications in the PIC group, compared to 18 (40%) in SHDU-plus. Mean specialist unit charge per day was less on SHDU-plus compared with PIC. There were no bed-shortage cancellations in the SHDU-plus group, compared to 11 in the PIC group. For children with neuromuscular or syndromic scoliosis undergoing PSF and deemed not suitable for post-operative care on the hospital floor, creation of a SHDU-plus was associated with fewer readmissions back to PIC or HDU, shorter hospital stays, an equivalent complication rate, significant cost-saving and fewer cancellations. Level of Evidence: Therapeutic Level III

Abstract. In the pediatric population, scoliosis is classified into congenital, syndromic, idiopathic, and neuromuscular in aetiology. Syndromic scoliosis represents a wide range of systemic anomalies associated with scoliosis. The primary challenge for a clinician is to think beyond the scoliotic curve, as the underlying pathology is multisystemic. The aim of this review is to identify the systemic anomalies, associated with syndromic scoliosis. MEDLINE, EMBASE, and CINAHL databases were searched, dating from 1990–2020, relevant to the purpose of our study. Keywords used: “scoliosis”+ “syndrome” + “genetic”. Retrospective, prospective studies were included. Case reports that had fewer than 4 patients were not included. Delineating 60 articles, we found a total of 41 syndromes to be associated with scoliosis. Thoracic region was the most common level of scoliosis curve, being noted in 28 syndromes. Mental retardation, seizures, and ataxia were the commonly noticed CNS anomalies. VSD, ASD, and TGA were the anomalies associated with CVS; Hypotonia, rib and vertebral malformations were the most identified neuromuscular anomalies; pulmonary hypoplasia, renal agenesis, and strabismus were other associations. A multidisciplinary approach, involving spinal surgeons, paediatricians, geneticists, anesthesiologists, and allied health professionals, is vital for the best care of patients with syndromic scoliosis. The location of the scoliotic curve reflects the associated anomalies, as thoracic curvature is more closely linked with cardiac anomalies, while lumbosacral curvature is seen to be often linked with genitourinary anomalies. We hope that this article provides a clear overview of the systemic associations in syndromic scoliosis and thus, facilitates and streamlines the management protocol

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine: if ultra-low dose CT without sedation was feasible given the movement disorders in this population; what the radiation exposure was compared to standard pre-operative imaging; whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α = 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine:. if ultra-low dose CT without sedation was feasible given the movement disorders in this population;. what the radiation exposure was compared to standard pre-operative imaging;. whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α= 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population