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

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

Background: The incidence of intra-spinal abnormality in congenital scoliosis is high. McMaster et al found an 18% incidence of myelographic abnormality in a series of 251 patients. Our objective was to report the MRI findings in a large series of patients with congenital scoliosis. Method: The notes, X-rays and MRI of 126 congenital scoliosis patients were reviewed to note the vertebral abnormality, curve progression, MRI findings and the presence of non-spinal congenital abnormality. These findings were then correlated to detect any association between them. Result: Forty-six patients (37%) had intra-spinal abnormalities detected on MRI. Sixty-six patients had failure of formation, 10 had failure of segmentation, 34 had mixed vertebral anomaly and 16 had congenital kyphosis or dislocation. MRI abnormality was significantly higher among patients with mixed anomaly (41%), congenital kyphosis (57%) and segmentation anomaly (40%) than those with failure of formation (29%). Presence of MRI abnormality did not correlate with curve progression or the presence of congenital abnormality affecting other organs. Conclusion: Intra-spinal abnormality in congenital scoliosis occurred in 37% cases. The incidence of such abnormality is higher in patients with congenital kyphosis, failure of segmentation and mixed vertebral anomalies

Background: Congenital anomalies of the chest wall and Sprengel’s shoulder are often associated with congenital deformities of the spine. It has been suggested that extensive rib fusions on the concavity of a congenital scoliosis may adversely affect progression of the spine deformity, thoracic function and growth of the lungs, which can lead to a thoracic insufficiency syndrome. Methods: This is a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital spine deformities. The rib anomalies were classified into simple and complex and the presence of a Sprengel’s shoulder was recorded. Subjects: There were 497 patients (80%) with scoliosis, 88 patients (14%) with kyphoscoliosis, and 35 patients with kyphosis (6%). The mean rate of scoliosis deterioration without treatment, age and curve size at surgery was compared for the different types of vertebral abnormalities in patients with and without rib anomalies. Results: Rib anomalies occurred in 124 patients. The rib anomalies were simple in 97 patients (78%) and complex in 27 (22%). The most common simple rib anomaly (70 patients; 72%) was a fusion of two or three ribs. The most common complex rib anomaly (20 patients; 74%) was a fusion of multiple ribs associated with a large chest wall defect. Rib anomalies were most commonly associated with a congenital scoliosis (116 patients; 93.6%), and much less frequently with a congenital kyphoscoliosis or kyphosis (8 patients). In those patients with a scoliosis, the rib anomalies were simple in 91 patients (78.5%) and complex in 25 patients (21.5%). Eighty-eight of the 124 patients (76%) with rib abnormalities had a scoliosis due to a unilateral unsegmented bar with or without contra-lateral hemivertebrae at the same level, and 8 patients had mixed unclassifiable vertebral anomalies. In contrast, only 17 patients with a scoliosis and rib anomalies had hemivertebrae alone. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (107 patients; 92.2%) and occurred on the concavity in 81 patients (70%), convexity in 27 patients (23%), and were bilateral in 8 patients. Sprengel’s deformity occurred in 45 patients; 43 patients with congenital scoliosis (8.6%), and 2 with kyphoscoliosis or kyphosis. A Sprengel’s shoulder occurred most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (28 patients). The elevated shoulder was on the concavity of scoliosis in 26 patients and on the convexity in 16 patients. We compared the mean rate of curve progression without treatment, the age and curve size at spine surgery for the different types of spine deformities in patients with and without rib anomalies either simple or complex and found no significant difference (p> 0.05); the only statistical difference was that the mean age at surgery was higher for patients with a unilateral unsegmented bar without rib anomalies (p=0.005). Conclusion: Congenital rib anomalies and Sprengel’s shoulder occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis due to a unilateral failure of vertebral segmentation. However, these anomalies do not have an adverse effect on curve size or rate of progression

Study Design: Retrospective study with clinical and radiological evaluation of 29 patients with congenital scoliosis who underwent 31 short segment anterior instrumented fusions of lateral hemivertebrae. Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital scoliosis in children below the age of 6. Summary of background data: A variety of treatments have been described in the literature for the treatment of HV. We report the results of a novel technique. Materials and Methods: Between 1996 and 2005, 29 consecutive patients with 31 lateral HV and progressive scoliosis underwent short segment anterior instrumentation and fusion with preservation of the HV. Mean age at surgery was 2.9 years. Mean follow-up period was 6.3 years. Results: Preoperative segmental Cobb angle averaging 39°, was corrected to 150 after surgery, being 15º at the last follow up (60% of improvement). Compensatory cranial and caudal curves corrected by approximately 50% and did not change significantly on follow up. The angle of segmental kyphosis averaged 13º before surgery, 12º after surgery, and 12° at follow up. There was 2 wound infection requiring surgical debridment, 1 intraoperative fracture of the vertebral body and 1 case lost correction due to implant failure. All went on to stable bony union. There were no neurologic complications. Conclusions: Early diagnosis and early and aggressive surgical treatment are mandatory for a successful treatment of congenital scoliosis and to prevent the development of secondary compensatory deformities. Anterior instrumentation is a safe and effective technique capable of transmitting a high amount of convex compression allowing short segment fusion which is of great importance in the growing spine

Background: Surgical treatments described for congenital spinal deformity are i) convex growth arrest, ii) posterior or combined anterior and posterior fusion and iii) hemivertebrectomy. Posterior instrumentation is used as an adjunct to fusion, whenever possible. Anterior instrumentation for correction of congenital scoliosis has not been described. A preliminary report of the use of anterior instrumentation following hemi-vertebrectomy for correction of congenital spinal deformity is reported. Method: 15 patients with congenital scoliosis and 5 patients with congenital kyphosis underwent hemiverte-brectomy and anterior instrumentation with fusion for single-stage correction of deformity . The average age of the patients at the time of surgery was 31 months and at last follow-up 59 months. All patients had pre-operative MRI. Twelve patients had normal and 8 had abnormal MRI. The average operating time was 135 minutes and average blood loss was 462 ml. Implants used were downsize Synergy, Orion Colorado and AO Cervifix. Average sagittal Cobb angle for the scoliosis patients was 45.5° pre-operatively and 16.8° post-operatively. Average coronal Cobb angle in patients with congenital kyphosis was 61° pre-operatively and 21° postoperatively. At an average follow-up of 17 months, the correction is well maintained in all except one. This patient developed pseudarthrosis at 19 months post-operatively. This was treated with posterior instrumented fusion. There were no cases of neurologic compromise or deep wound infection. Conclusion: Because of the young age at which hemiver-tebrectomy is performed in congenital scoliosis patients, instrumentation is difficult. Posterior instrumentation has been well described in literature. Our early experience with anterior instrumentation after hemivertebrectomy shows promising results with very good correction of the deformity and no increase in complication rate

Study design: A retrospective review of patient records with recent clinical and radiographic assessment. Objective: Long-term evaluation of the Luque trolley for posterior instrumentation in congenital scoliosis. Summary of background data: From a group of 51 cases treated with the Luque trolley, we review 10 patients with progressive congenital scoliosis (5male, 5female) for a mean follow-up period of 14.8 years, to mean age of 19 years. The mean Cobb angle of the primary curve before surgery was 69.5 degrees. The mean Cobb angle of the secondary cervico-thoracic curve before surgery was 37.1 degrees and of the caudal secondary curve was 26.4 degrees. The mean age at surgery was 5.0 years. 8 patients had a selective epiphysiodesis procedure, 2 with hemi-vertebrectomy, and all underwent single- stage (7 patients) or dual-staged (3 patients) posterior instrumentation with a Luque trolley growing construct. Method: Clinical evaluation and sequential measurements of Cobb angle were done, with recording of further surgical procedures, associated complications, and final coronal balance. The thoracolumbar longitudinal spinal growth (T1-S1) and growth in the instrumented segmented were also calculated. Results: The mean preoperative primary curve Cobb angle of 69.5degrees, corrected to a mean postoperative angle of 30.6 degrees, with progression from here to curve magnitude of 38.8 degrees on latest follow up (approximate rate of progression of 0.55 degrees per year). The mean pre-operative cephalic (cervico-thoracic) Cobb angle of 37.1degrees, corrected to 22 degrees, with progression to 26.6 degrees. The mean pre-operative caudal (lumbar) Cobb angle of 26.4degrees, corrected to16.2 degrees, this later progressed to 20.6 degrees. Coronal plane translation measured 1.68 cm at latest follow up [range 0.5–5.1cm]. The thoracolumbar longitudinal growth measured a mean of 8.81cm (approx0.8 cm/year) with a recorded lengthening of 2.54 cm (approx 0.23cm/year) in the instrumented segmented. Half the patients did not require further surgery. Conclusion: Selective fusion does not always prevent further deformity in congenital scoliosis. The addition of posterior growing construct instrumentation did demonstrate capacity for good correction of primary and secondary curvatures and a limited capacity for further longitudinal growth

Cervico-thoracic congenital scoliosis is a difficult deformity to obtain good correction due to its anatomical characteristics and lack of proper instrumentation. Surgical treatments often end up with poor correction by convex epiphysiodesis alone, making hideous residual head tilt. This is a report of 2 cases with cervico-thoracic congenital scoliosis, which underwent total excision of hemivertebra, instrumentation and fusion through posterior approach alone. Case 1. 8y2m old Girl who had T1, T3, T7 hemivertebrae with a left convex curve from C7 to T11. At age 5. she had tilted head and left convex 33 degrees scoliosis. Only regular observation was done. At age 8y2m, the scoliosis had progressed to 49degrees. Total excision of T1 hemivertebra was performed. At age 10y8m, total excision of T7 hemivertebra, extension of instrumentation and fusion to T10 was performed. These procedures brought almost normal alignment on both sagittal and coronal plane. However, lower compensatory curve progressed later on, fusion was extended to L2 at age 13 resulting in excellent balance. Case 2: Girl. 2y7m. Multi-level hemivertebrae. C6-L1 L100 degrees. Total excision of T12 hemi, short fusion and instrumentation reduced the scoliosis to 50 degrees. Five months later, total excision of T9 hemi was done. Four months later, concave side instrumentation from T2 to L2 without fusion was done. At age 4y2m, total excision of T1 hemi was done using cervical pedicle screw. The scoliosis is being controlled at 35 degrees with one extension of the rod later on

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

Purpose: The risk of injuring the radial nerves during spine instrumentation to correct spinal deformity is well known and accounts for about 50% of the neurological complications associated with this type of surgery. We describe a technique for monitoring the nerve roots during spinal surgery. Radicular monitoring was described by Hormes in 1993. Material and methods: We report a retrospective analysis of 73 procedures for spinal deformity during which the nerve roots were monitored. The series included 27 men and 46 women, mean age 23.9 years (range 4.5–74.9). Forty patients were less than 18 years old. Procedures included posterior arthrodesis (n=65) and anterior arthrodesis (n=8). Indications were: idiopathic scoliosis (n=32), neurological scoliosis (n=21), congenital scoliosis (n=4), spondylolisthesis (n=2) and kyphosis (n=3). The study group included 68 patients (168 roots) with recordings obtained under the required conditions. The routine procedure involved permanent electrophysiological monitoring of muscle activity with a multi-channel electromyograph. We used microwires implanted within the muscle itself for electrodes. Target muscles depended on the position of the planned implants and the topography of the roots likely to be endangered during the surgical procedure or instrumentation. Explored roots were: T12 (n=9), L1 (n=24, L2 (n=40), L3 (n=24), L4 (n=23), L5 (n=11), S1 (n=22). Monitoring prohibited use of curare during anaesthesia. Results: Prior to radicular monitoring, we had had two root injuries (T12 and L3) which resolved spontaneous (n=139). During the study, changes in the radicular signal were observed in seven patients. All signal anomalies triggered a modification of the surgical procedure and no postoperative deficit was observed. Incidents observed concerned congenital scoliosis (n=2), neurological scoliosis (n=2), and idiopathic scoliosis (n=3). Roots involved were L1 (n=1), L2 (n=2), L3 (n=2), L4 (n= 4), i.e. 11/163. Discussion: Continuous intraoperative monitoring of the spinal roots exposed to surgery for spinal deformity enabled us to identify eleven cases of root suffering among 163 recordings. This permanent monitoring system enabled us to immediately modify the surgical procedure and to control and conflict between the instrumentation and the roots or possible stretching during the correction. This technique requires permanent monitoring during the spinal procedure to avoid false negatives. Curare cannot be used. Conclusion: Intraoperative radicular monitoring is an effective way to avoid radicular complications of this type of surgery. The technique is sensitive and allows immediate adaptation of the surgical procedure. It requires close collaboration between the neurophysiologist, the orthopaedic surgeon and the anaesthesiologist

There can be no doubt that bone morphogenetic proteins play a hierarchic role in the osteogenetic cascade. Pre-clinical and clinical trials have confirmed their decisive role in achieving anterior lumbar fusion, as they direct mesenchymal stem cells toward osteoblastic lineages.The present study is concerned with initial experience in the application of autologous mesen-chymal stem cells and various growth factors (BMP-7,VEGF,TGFbeta) in the treatment of paediatric spinal pathologies. Eleven patients affected by serious forms of congenital infantile scoliosis, idiopathic scoliosis and grade I spondylolisthesis received surgical treatment. In three patients with congenital infantile scoliosis, ages ranging from 3 to 12 years, the surgical procedure was an anterior and posterior fusion at the level of the hemiver-tebra, extending it to a level above and below it by means of in situ decortication of the vertebral plates and laminae on the convex side and delivery of stem cells taken from the iliac bone and applied in situ by means of bovine collagen sponge (Healos system). No fixation device was added. Plaster and brace were used during the postoperative course for 9 months. In two cases of intertransverse in situ fusion for grade I spondylolisthesis the age of the patients was 13 and 16 years, respectively, and the surgical procedure consisted in the standard technique to which was added delivery of a mixture composed of small bone chips obtained from decortication, 3.5 mg of eptotermin alpha (Op-1, BMP7), and autologous stem cells taken from iliac bone. A special form of informed consent was obtained for these two patients because of their incomplete bone maturity. TLSO was used in the postoperative course for 2 months. In the six patients with idiopathic scoliosis, ages ranged from 13 to 15 years and the treatment consisted in posterior instrumentation and fusion by means of rods, transpedicular screws and hooks; standard fusion techniques were supported by local bone chips obtained from decortication, placed on collagen sponges and combined with autologous stem cells taken from iliac bone with the addition of platelet gel derived from the autologous preoperative blood collection. No bone chips were taken from iliac wing. The results were evaluated by X-rays and CT at intervals of 1, 2, 4, 6 and 12 months. In the cases of congenital scoliosis a solid fusion area was obtained only for posterior hemiephysiodesis, without a parallel bone signal of fusion at the anterior level. In the cases of intertransverse in situ fusions for spondy-lolisthesis there was a beginning fusion already visible on the first X-ray control 1 month postopoeratively, confirmed at successive check-ups and maintained in the follow-up. The cases of idiopathic scoliosis showed an initial ossification of the grafts and signals of fusion at the 6-month check-up. The isolated use of stem cells, although promising from a theoretical point of view, did not prove encouraging in the cases of anterior fusion. It is most probable that the absence of instrumentation induced a defect in the stable fixation of the fused segments, the latter being a crucial factor.The cases of in situ fusion for spondylolisthesis confirm the hierarchic role of the bone morphogenetic protein 7 in inducing the mesenchymal stem cells, released in situ from decortication, toward osteoblastic lineage. To our knowledge these represent the first two cases of use of OP-1 in patients younger than 18 years. In the fusion areas which are more extensive in length (idiopathic scoliosis) the added use of autologous stem cells mixed with platelet gel seems to improve the physiological processes of fusion. It will be necessary to monitor the long-term results of these procedures with special regard to loss of correction and weakness of the fusion area causing torsional or flexion-extension stress. These possible costs have to be compared with the verified benefits of a better use of blood in its capacity to save on corresponding haemotransfusion, combined with the absence of comorbidity related to the donor site of iliac bone chips

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

The purpose of this study was to ascertain whether early diagnosis through antenatal ultrasound screening and intervention lead to a reduction in morbidity associated with congenital scoliosis. Prospective cohort study. All fetuses with vertebral body anomalies detected over a thirteen years period were included. Maternal risk factors, accuracy of antenatal diagnosis, associated fetal anomalies and ultimate outcome are described. Twenty-four fetuses with congenital hemivertebrae were identified from 39,000 antenatal scans (incidence 0.061%). The mothers' median age at conception was 26 years (range 18-40 years). The median fetal gestational age at diagnosis was 20 weeks (range 18-38 weeks). The median gestational age at delivery was 38 weeks (range 27-40). Antenatal ultrasound identified eighteen fetuses with a single hemivertebra, six fetuses with multiple hemivertebrae. Eleven patients (50%) have undergone early surgical intervention with median post-natal follow up was 4 years (range 0-9 years). The median age at surgery was 12 months (range 1.2 - 47 months). The median pre-operative Cobb angle was 33 degrees and the median Cobb angle at final follow up was 25 degrees. In this series 50% of patients required surgical intervention. We felt that pre-natal diagnosis of congenital vertebra abnormally has resulted in early surgical intervention using less complex surgical technique to halt progressive deformity. However, further studies are required to compare the results with the abnormally not detected antenatally

Objective. Posterior vertebral column resection (PVCR) is indicated in the management of severe rigid spine deformities. It is a complex surgical procedure and is only performed in a few spine centres due to the technical expertise required and associated risk. The purpose of this study is to review the indications, surgical challenges and outcomes of patients undergoing PVCR. Methods. 12 patients with severe spinal deformities who underwent PVCR were retrospectively reviewed after a follow-up of 2 years. Surgery was performed with the aid of motor evoked spinal cord monitoring and cellsaver when available. The average surgical duration was 310 minutes (100–490). The average blood loss was 1491 ml (0–3500). The indication for PVCR was gross deformity and myelopathy which was due to congenital spinal deformities and one case of old tuberculosis. Clinical records and the radiographic parameters were reviewed. Results. Kyphosis of an average of 72 degrees was corrected to 28 degrees. The associated scoliosis was corrected from an average of 49.2 to 21.2 degrees. Ten patients improved neurologically to ASIA D and E. One patient deteriorated markedly, required revision with no initial improvement but reached ASIA E at 6 months after surgery. Four patients had associated syringomyelia. All were re-scanned at 1 year. The three with small syrinx's demonstrated no progression on MRI and the large syrinx resolved completely. In addition to the neurological deterioration, complications included 1 right lower lobe pneumonia. Conclusion. PVCR is an effective option to correct complex rigid kyphoscoliosis. In addition it allows excellent circumferential decompression of the cord and neurological recovery. When the congenital scoliosis is associated with syringomyelia with no other cause evident, it may allow resolution of the syrinx. Key words: Posterior vertebral column resection, severe spinal deformities, myelopathy, syringomyelia. NO DISCLOSURES

Study Design. An observational study in patients with congenital spinal deformity. Objective. To review the relationship between hemivertebrae and the posterior spinal elements in congenital spinal deformity. Summary of background data. The vertebral body anomalies of congenital scoliosis have been classified and their natural history is well known. More recently a variety of posterior element anomalies have been classified. The relationship between posterior and anterior structures is important when posterior excision of a hemivertebrae is undertaken. Methods. Patients recorded as having congenital spinal deformity were identified from a database. All available imaging was obtained and reviewed with particular reference to posterior element anatomy as visualised on CT or MRI scans. Results. In total 36 patients with congenital spinal deformity were identified and their imaging was reviewed. 29 hemivertebrae were identified in 24 patients. Clear images were obtained of the posterior elements in 18 cases. 9 isolated hemilaminae (as visualised on coronal CT or MRI) were seen in 8 patients with associated hemivertebrae. In 7 of 9 cases the hemilamina was found to be one level cranial to the hemivertebrae and in the remaining 2 cases it was at the same level. Conclusion. When planning excision of a hemivertebra, whether this is performed by a posterior approach or by combined anterior and posterior surgery, care must be taken to avoid wrong level surgery. Pre-operative assessment with CT and MRI scans is essential. The surgeon must be aware of the high possibility that the posterior element anomaly may be proximal to the vertebral body anomaly both when considering how to excise the hemivertebra and for planning the fixation levels. Statement of interests: The authors declare that they have no competing interests

Purpose. To report the results of full vertebral column resection (VCR) for paediatric spinal deformity. Methods and Results. All VCR (n=47) for paediatric spinal deformity were retrospectively evaluated from four university hospitals performing these procedures in Finland between 2005 and 2010. After excluding single hemivertebra (n=25) and resections performed for patients with MMC (n=6), 16 patients with full VCR (mean age at surgery 12.9 yrs [6.5-17.9] AIS 1; NMS 3; Congenital scoliosis 3 primary, revision 4; Kyphosis congenital 2, global 2; NF1 scoliosis 1) were identified. Seven procedures were performed anteroposteriorly and nine posterior-only. Mean follow-up time 1.9 (0.6–5.5) years. Major Curve (MC) averaged preoperatively 85 (58–120) degrees, 31 (14-53) degrees at 6 months, and 37 (17-80) degrees at 2-year follow-up. MC correction averaged 61 (46-86)% in the AP and 64 (57-83)% in the PL group at 6 months and 54 (18-86)% and 60 (41-70)% at 2-yr FU, respectively (NS). Blood loss averaged 3400 (500-8200) mL (NS between groups). The mean SRS-24 total scores were 100 (92-108) for AP and 102 (95-105) for PL group. There was one paraparesis in the AP group necessitating urgent re-decompression with full recovery. One peripheral L5 motor deficit resolved fully within few days (PL). Two junctional kyphosis were observed (one in both group). One one-sided partial lower instrumentation pull-out was observed without need for revision. One pseudoarthrosis occurred in AP group needing revision. Conclusions. Full VCR is rarely needed for paediatric spinal deformity with an estimated incidence of 2.9/million/year. Posterior VCR allows better control of neural elements during deformity correction

Purpose: Prediction of progression is actually impossible in adolescent idiopathic scoliosis (AIS). Potential risk factor to consider at first visit might be morphologic parameters of the spine. The objective of this study was to compare 3D morphologic parameters of the spine in a non evolutive an in an evolutive group of AIS. Method: A retrospective cohort study was done. Two groups were recruited with sample size based on a difference of 5 degrees for rotation parameters. First group were all surgical patients (n=19) and second group non evolutive patient (n=18). Inclusion criteria were. Risser sign of 0 or 1. Cobb angle between 11 and 40 degrees. AP and lateral radiograph available. Exclusion criteria were. limb length discrepancy. syndromic or congenital scoliosis. All spines were reconstructed in 3D with AP and lateral radiographs of the first visit and measurements were performed on the reconstruction. There were 4 categories of measurements done: Cobb angle, wedging, rotation, slenderness. Student t test were performed. Results: There was no statistical difference between the two groups for Cobb angle in maximal plane, for lordosis and kyphosis. Differences were found for wedging of the apical disk in 3D plane (S=5,4° vs NE= 0,7° with p=0,04). For coronal orientation of the apex (S=7,8° vs NE=0,1° with p=0,01). For axial orientation of inferior junctional vertebrae (S=1,9° vs 0,1° with p=0,007). For torsion (S=−4,1° vs NE= – 1,2° with p=0,03). For ratio between height and width of T6 (S=51% vs NE=53,6% with p=0,04). Conclusion: This study give for the first time some 3D morphologic parameters that could be use in the prediction of AIS. Some limitations exist such as the small sample size and the low level of significance. In the future those parameters will be used in the development of a prediction model base on those keys parameters that will confirm the actual findings

Beighton and Kozlowski, in 1980, first defined this disorder, in Afrikaners, the syndrome , evident at birth, is constantly manifested by dwarfism, ligamentous hyperlaxity, congenital scoliosis, and multiple dislocations(hip dislocation, radial head dislocation, scoliosis, spatulate thumbs, and generalized ligamentous hyperlaxity, children are of normal intelligence potential. We report A Tunisian family, in which the proband , and her parents family have the presumptive diagnosis of Beighton dysplasia(spondyloepimetaphyseal dysplasia), the proband manifested the full clinical criteria of the disorder, whereas the mother, and other family members are partially manifesting the disorder, but dysplastic hips is the common feature in most of the family members examined(from both paternal and maternal side), up to four family subjects are variably affected, ranging between congenital hip dislocation in two subjects and post adulthood dysplastic arthrosis. The striking clinical findings evolved from the study is the accompaniment of diverse skeletal abnormalities rather than the hip dysplasia, the mother is with adolescent type kyphosis, and two other family subjects are with short stature and scoliosis, fractures were encountered in three family subjects, this was secondary to osteoporosis, which in fact a general clinical feature in all the family subjects. The mode of inheritance of the disorder is compatible with autosomal recessive trait. The purpose of this study is to indicate the importance of the precise recognition of the underlying etiologies in children presented with congenital dysplastic hips, as a matter of fact this is our main strategy in the department

Aims

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 study aimed to develop and assess a new classification system for Segmentation Defects of the Vertebrae (SDV), a frequent cause of congenital scoliosis. Existing nomenclature for the wide range of SDV phenotypes is inadequate and confusing eg ‘Jarcho-Levin syndrome’. A multidisciplinary group of the International Consortium for Vertebral Anomalies and Scoliosis (ICVAS) met to formulate a new classification system, based primarily on radiology. SDV are identified by number affected, contiguity, and spinal region(s). The size, shape and symmetry of the thoracic cage, and rib number, symmetry and fusion are included, and familiar vertebral morphology terms retained, together with accepted syndrome names. The terms spondylocostal and spondylothoracic dysostosis apply only to phenotypes typified by the monogenic disorders due to mutated DLL3, MESP2, LNFG and HES7 genes. Five ICVAS members (Group 1) then independently assessed 10 new cases, inter-observer reliability assessed using kappa. Seven independent radiologists (Group 2) then assessed the same cases before and after introduction to the new system. Inter-observer reliability for Group 1 yielded a kappa value of 0.21 (95% confidence intervals (CI) 0.052, 0.366, p=0.0046). For Group 2, before introduction to the new system, 1/70 responses (1.4%) agreed with the Group 1 consensus,12 different diagnoses were offered, and 38/70 (54.3%) responses were ‘Unknown’. After introduction to the new system 47/70 responses (67.1%; 95% CI 55.5, 77.0) agreed with Group 1 consensus, a 65.7% improvement (95% CI 52.5, 75.6, p< 0.00005). The system was well received by 6/7 radiologists. The new system was found to be reliable and acceptable. Ethics approval: None. Interest Statement: None