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

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In this investigation, we administered oxidative stress to nucleus pulposus cells (NPCs), recognized DNA-damage-inducible transcript 4 (DDIT4) as a component in intervertebral disc degeneration (IVDD), and devised a hydrogel capable of conveying small interfering RNA (siRNA) to IVDD.

The August 2023 Children’s orthopaedics Roundup. 360. looks at: DDH: What can patients expect after open reduction?; Femoral head deformity associated with hip displacement in non-ambulatory cerebral palsy; Bony hip reconstruction for displaced hips in patients with cerebral palsy: is postoperative immobilization indicated?; Opioid re-prescriptions after ACL reconstruction in adolescents are associated with subsequent opioid use disorder; Normative femoral and tibial lengths in a modern population of USA children; Retrospective analysis of associated anomalies in 636 patients with operatively treated congenital scoliosis; Radiological hip shape and patient-reported outcome measures in healed Perthes’ disease; Significantly displaced adolescent posterior sternoclavicular joint injuries

The April 2023 Children’s orthopaedics Roundup³⁶⁰ looks at: Can you treat type IIA supracondylar humerus fractures conservatively?; Bone bruising and anterior cruciate ligament injury in paediatrics; Participation and motor abilities after treatment with the Ponseti method; Does fellowship training help with paediatric supracondylar fractures?; Supracondylar elbow fracture management (Supra Man): a national trainee collaborative evaluation of practice; Magnetically controlled growing rods in early-onset scoliosis; Weightbearing restrictions and weight gain in children with Perthes’ disease?; Injuries and child abuse increase during the pandemic over 12,942 emergency admissions.

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.

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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.

SUMMARY. A retrospective cohort study of 19 patients of EOS who underwent MCGR rod instrumentation with subsequent serial distractions, measured using ultrasonography. The degree of distraction achieved during each session were analyzed and subgroup analysis done. HYPOTHESIS. The degree of distraction/ distractibility of the MCGR rod is closely linked to the etiology of EOS. The degree of distraction achieved at each session progressively diminishes with successive distractions. STUDY DESIGN. Retrospective cohort study. INTRODUCTION. MCGR rods have gained popularity as an alternative to traditional growing rods for the treatment of Early-onset scoliosis (EOS), serially distracting with an aim to mimic the normal spinal growth, which can be achieved in an outpatient setting using external remote controller. The use of ultrasound as a tool to measure the degree of distraction achieved has been validated previously. However the association between the etiological diagnosis of EOS and the degree of distraction achieved has not been studied previously. METHODS. We performed a retrospective cohort study of 19 patients with EOS of varying etiologies who underwent MCGR rod instrumentation at our institution since 2016. Their hospital records were analyzed to assess the demographics, distraction interval and the degree of distraction achieved at each session. RESULTS. 19 patients (12 females and 7 males) were included in the study. The average age of the study population was 7 years (4–13 years). The average follow up was 2.1 years (1–4). The following were the etiological diagnoses: Idiopathic EOS(8), Neuromuscular scoliosis(5), Syndromic(5), Congenital(1). The average interval between each distraction was 109.8days (88.2–140.6). The overall mean distraction was 1.66mm (0.6–3.427). The mean distraction achieved for the concave rod was 1.69mm (0.6–3.03) as against 1.59mm (0.2–3.427) for the convex rod. The degree of distraction achieved per session was the greatest in patients with neuromuscular scoliosis 2.79mm (0.94–4.62), while it was the least in Congenital scoliosis 0.99mm (0.8–2.93). The degree of distraction in Syndromic and Idiopathic scoliosis groups were 2.19mm (0.1–4.2) and 1.50mm (0.2–4.45) respectively. The mean distraction achieved during the first session after MCGR instrumentation was 2.82mm (0.4–9.8) as against 1.98mm (0.1–4.2) and 1.18mm (0.2–2.3), achieved during the 5. th. and 10. th. distractions respectively. CONCLUSIONS. The average distraction of the MCGR rod achieved per session depends upon the etiological diagnosis of EOS. Neuromuscular curves are the most amenable to MCGR distractions, while the congenital curves are the least. The degree of distraction achieved progressively diminishes with each successive distractions. The distraction achieved slumps to 70% of the initial distraction by the 5. th. session and further declines to 41% of the initial distraction by the 10. th. session. TAKE HOME MESSAGE. The distractibility of the MCGR rod is closely linked to the etiology of EOS. MCGR rods are most effective in cases of neuromuscular scoliosis, while least effective in congenital scoliosis. The law of diminishing returns holds true even with MCGR rods

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To benchmark the radiation dose to patients during the course of treatment for a spinal deformity.

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

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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).

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To investigate metallosis in patients with magnetically controlled growing rods (MCGRs) and characterize the metal particle profile of the tissues surrounding the rod.

Intrathecal morphine (IM) is a common adjunct in paediatric spinal deformity surgery. We previously demonstrated with idiopathic scoliosis it provides safe and effective analgesia in the immediate postoperative period. This study represents our 25 year experience with IM in all diagnostic groups. Our prospective Pediatric Orthopaedic Spine Database (1993–2018) was reviewed to identify all patients undergoing spinal deformity surgery who received IM and who did not. Patients 21 years of age or less who had a posterior spinal fusion (PSF) with segmental spinal instrumentation (SSI), and received 9–19 mcg/kg (up to 1 mg) of IM were included. Early onset scoliosis surgical patients were excluded. We assessed demographics, pain scores, time to first dose of opioids, diagnoses, surgical time, paediatric intensive care unit (PICU) admission and IM complications (respiratory depression, pruritus, nausea/ vomiting). There were 986 patients who met inclusion criteria. This included 760 patients who received IM and 226 who did not. IM was not used for short procedures (< 3 hrs), respiratory concerns, unsuccessful access of intrathecal space, paraplegia, and anesthesiologist decision. Both groups followed the same strict perioperative care path. The patients were divided into 5 diagnostic groups (IM / non IM patients): idiopathic (578/28), neuromuscular (100/151), syndromic (36/17), and congenital scoliosis (32/21) and kyphosis (14/9). Females predominated over males (697/289). The first dose of opioids after surgery was delayed for a mean of 10.6 hrs in IM group compared to 2.3 hrs in the non-IM group (p=0.001). The postoperative pain scores were significantly lower in the IM groups in the Post Anesthesia Care Unite (p=0.001). Only 17 IM patients (2%) were admitted to the PICU for observation secondary to respiratory depression, none required re-intubation. None of the IM group were re-intubated. Forty-nine patients (6%) experienced pruritus in the IM group compared to 4 of 226 patients (2%) in the non IM group. There were 169 patients (22%) of the IM patients and 21 patients (9%) of the non IM had nausea and vomiting postoperatively. Three patients (0.39%) had a dural leak from the administration of IM but did not require surgical repair. There were no other perioperative complications related to the use of IM. There were no significant group differences. Pre-incision IM is a safe and effective adjunct for pain management in all diagnostic groups undergoing spinal surgery. The IM patients had lower pain scores and a longer time to first administration of post-operative opioids. Although there is an increased frequency of respiratory depression, pruritus, and nausea/vomiting in the IM group, there were no serious complications

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Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

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Magnetically controlled growing rod (MCGR) systems use non-invasive spinal lengthening for the surgical treatment of early-onset scoliosis (EOS). The primary aim of this study was to evaluate the performance of these devices in the prevention of progression of the deformity. A secondary aim was to record the rate of complications.

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Magnetically controlled growing rods (MCGRs) allow non-invasive correction of the spinal deformity in the treatment of early-onset scoliosis. Conventional growing rod systems (CGRS) need repeated surgical distractions: these are associated with the effect of the ‘law of diminishing returns’.

The primary aim of this study was to quantify this effect in MCGRs over sequential distractions.

Introduction. Originally, the vertical expandable titanium rib (VEPTR™) was developed to treat children with Thoracic insufficiency syndrome secondary to fused ribs and congenital scoliosis. Over the years its usage has widen and is currently being used to treat all etiology of early onset scoliosis (EOS). A major draw back remains the size of the titanium VEPTR™ implant. In keeping with the new trend of chrome-cobalt alloy (CoCr). spinal implants, we set out to explore if redesigning the VEPTR™ was mechanically sound. The aim of this study was twofold. Firstly, we investigate the mechanical properties of a VEPTR™ made with CoCr alloy compared to that of titanium alloy. Secondly we investigated how much we could down size the VEPTR™. Materials & Methods. Finite element analyses were performed on 3 different VEPTR™ designs (rod diameter of 6mm, 5mm and 4mm) subjected to a compressive load of 500N (equivalent to a 50Kg child). For each configuration, two materials, titanium alloy and chrome-cobalt alloy, were used. Maximum Von Mises stress distribution (VMSD), plastic strain (PS) and total displacement (TD) of the VEPTR™ were measured as indicators of mechanical properties of the implant. Results. Results for the maximum Von Mises stress distribution (VMSD), plastic strain and total displacement (TD) can be seen on the table 1. Discussion. Results confirm that yield strength of titanium material is greater than that of Co-Cr, while Plastic strain (PS) is greater for a CoCr VEPTR™ than for titanium VEPTR™. As expected a 6 mm CoCr VEPTR resist displacement almost twice as a 6 mm titanium VEPTR. Little difference is noted in plastic strain and VonMises stress at 6mm. Down sizing the implant to 5 mm in titanium or CoCr may runs the risk of implant failure as both exceeds their failure point and they both deform 0.29% and 6.6% respectively, placing the 5mm CoCr at higher risk of failure. Our results suggest that the VEPTR™ design could be reduced to 5mm however requires a new design to minimize the risk of failure. 4mm rods will not withstand a 50kg load

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We reviewed 34 consecutive patients (18 female-16 male) with isthmic spondylolysis and grade I to II lumbosacral spondylolisthesis who underwent in situ posterolateral arthodesis between the L5 transverse processes and the sacral ala with the use of iliac crest autograft. Ten patients had an associated scoliosis which required surgical correction at a later stage only in two patients with idiopathic curves unrelated to the spondylolisthesis.

The SRS-22 instrument was developed to assess quality of life in adolescent idiopathic scoliosis patients (AIS). The aim of our study was to evaluate the impact of surgical treatment on quality of life in neurologically normal patients with complex spinal deformity of a range of aetiologies using the SRS-22 questionnaire. We analysed prospectively collected SRS-22 data on 407 consecutive patients (315 females-92 males) treated from 2006–2012 under the care of the senior author. 97.6% of patients had a posterior (PSF), 1.3% an anterior (ASF) and 1.1% an anterior-posterior (A/PSF) spinal fusion. Questionnaires were completed at 4 stages of treatment: preoperatively, 6-months, 12-months and 24-months postoperatively. Other variables included age at surgery, gender, diagnosis and year of surgery. 9 diagnoses were included in the study: 271 patients had AIS; 39 Scheuermann's kyphosis; 31 spondylolisthesis; 16 congenital scoliosis; 13 JIS; 6 IIS; 13 scoliosis associated with intraspinal anomalies; 11 syndromic scoliosis; 7 scoliosis associated with congenital cardiac disease. Mean age at surgery was 15.14±2.07 years. Age at surgery was divided in 3 groups to compare outcomes against previous studies: 10–12, 13–15, and 15–19 years. Mean total SRS-22 scores for the whole group were: preoperative 3.62±0.66; 6-month 4.12±0.44; 12-month 4.39±0.40; and 24-month 4.52±0.37 (p<0.0001). Individual preoperative scores for the whole group were: function 3.77±0.75; pain 3.7±0.97; self-image 3.14±0.66; mental 3.86±0.77. Mean 24-month postoperative scores for the whole group were: function 4.39±0.42; pain 4.59±0.56; self-image 4.39±0.51; mental 4.43±0.56; satisfaction 4.81±0.40. All changes observed at 2-year follow-up were statistically significant (p<0.0001). Male preoperative total score (3.64±0.66) did not have a significant difference compared to female (3.62±0.66); however 24-month total score improvement was significant, with males (4.62±0.25) performing better than females (4.49±0.39) including satisfaction (p=0.004). Spondylolisthesis patients performed worse preoperatively (2.93±0.26) compared to other diagnoses (p<0.0001); AIS 3.67±0.64, congenital 3.81±0.57, syndromic 3.80±0.54, Scheuermann's 3.48±0.75, JIS 3.90±0.63, scoliosis with congenital cardiac disease 4.04±0.41 or intraspinal anomalies 3.71±0.66, and IIS 3.58±0.80. No change in total and individual scores was observed at 24-month review across diagnoses (p>0.05). There were no significant changes between each year of surgery in the study period and the total values at each stage (p>0.05). There were no significant changes between the 3 age ranges at each stage (p>0.05). Patients undergoing ASF performed significantly better at preoperative function (4.7±0.27) compared to PSF (3.76±0.74) and A/PSF (3.68±0.72), (p=0.19). All values at 2-years were not significant in comparison regarding type of operation (p>0.05). All individual domains and total SRS-22 scores improved after surgical correction of spinal deformity in our young patient cohort with an incremental change between preoperative, 6-month, 12-month and 24-month postoperative. Our 24-month postoperative outcomes in all diagnoses compare favourably to reported SRS-22 scores in healthy adolescents (function: 4.31±0.54; pain: 4.44±0.67; image: 4.41±0.64; mental health: 3.96±0.81; total 4.26±0.54). We are planning to continue this study with longer follow-up in order to allow comparison of our SRS-22 outcomes after surgical treatment to those obtained in patients treated with bracing or patients who were followed untreated (previous reports SRS-22 total scores: 4.2 for braced patients and 4.1 for observed patients)