Study Design. Retrospective review. Objective. To report the technique and results of vertebral column decancellation (VCD) for the management of sharp angular spinal deformity. Summary of Background Data. The goal of management of sharp angular spinal deformity is to realign the spinal deformity and safely decompress the neurological elements. However, some shortcomings related to current osteotomy treatment for these deformities are still evident. Methods. From January 2004 to March 2007, 45 patients (27 males/18 females) with severe sharp angular spinal deformities at our institution underwent VCD. The diagnoses included 29 congenital kyphoscoliosis and 16 Pott's deformity. The operative technique included multilevel VCD, disc removal, osteoclasis of the concave cortex, compression of the convex cortex accompanied by posterior instrumentation with pedicle screws. Preoperative and postoperative radiographic evaluation was performed. Intraoperative, postoperative and general complications were noted. Results. For a kyphosis type deformity, an average of 2.2 vertebrae was decancellated (range, 2to 4 vertebrae). The mean preoperative kyphosis was +98.6° (range, 82° to 138°), and the meankyphosis in the immediate postoperative period was +16.4° (range, 4° to 30°) with an averagepostoperative correction of +82.2° (range, 61° to 124°). For a kyphoscoliosis type deformity, thecorrection rate was 64% in the coronal plane (from 83.4° to 30.0°) postoperatively and 32.5°(61% correction) at 2 years follow-up. In the sagittal plane, the average preoperative curve of88.5° corrected to 28.6° immediately after surgery and to 31.0° at 2 years follow-up. All patientshad solid fusion at latest follow-up. Complications were encountered in 8 patients (17.8%) andincluded transient neurological deficit and complete paralysis (n = 1). Conclusion. Single stage posterior vertebral column decancellation (VCD) is an effective option to manage severe sharp angular spinal deformities

Introduction. Kyphoscoliosis is defined by a structural lateral curvature of the spine of 10° or more and an excessive thoracic kyphotic curve of 40° or more. Genetic analyses of families in which two or more members had kyphoscoliosis identified a 3·5 Mb area on chromosome 5p containing three genes of the Iroquois (IRX) homeobox family, IRX1, IRX2, and IRX4, which were then sequenced. Methods. Exons and highly conserved non-coding regions (HNCRs) 500 kb upstream and downstream fromIRX1, IRX2, and IRX4 were sequenced in 46 individuals from six families. Selection of these elements was based on PhastCons Placental Mammal Conserved Elements, Multiz Alignment. Single-nucleotide polymorphism (SNP) genotypes and sequence variants were obtained from all individuals. There were 431 SNPs, 61 in IRX4 regions, 80 in IRX2 regions, and 290 in IRX1 regions. 137 SNPs were novel. Mendelian inconsistencies were detected with PEDCHECK (inconsistency rate: 1·4%; missing data: 2·8%). SNPs and individuals with greater than 10% missing rate were excluded. Association analyses (ASSOC [SAGE version 6.0.1]) of the quantitative trait with patient's largest curve, were undertaken on 391 SNPs. Results. Association analyses resulted in 12 SNPs with p values less than 0·025, 11 of which were located upstream and downstream from IRX1. The most significant p value (p=0·000382) was obtained for rs35710183 (table). Multiple variants were found surrounding IRX1. The most prominent is a single base-pair deletion in all affected individuals genotyped in one family. All individuals with kyphoscoliosis and those with scoliotic curves greater than 35° had genotypes differing from the reference (unaffected) genotype for 23 SNPs. Several of these SNPs had significant p values for the association analyses done previously. Conclusions. The phenotype of kyphoscoliosis has been linked to sequence variants that lie within regulatory regions of the IRX homeobox gene family. Further analyses to establish the relevance of these findings will be done through in-vivo and in-vitro assays. The identification of spinal genetic determinants related to axial growth and maturation will help with the understanding of spinal pathology and potentially allow for development of directed therapeutic interventions

Introduction and Objective. Pectus carinatum is a common congenital anterior chest wall deformity, characterized by outward protrusion of sternum and ribcage resulted from rib cartilage overgrowth. The protrusion may be symmetrical or asymmetrical. Pectus carinatum association with mitral valve diseases, Marfan's syndrome, and scoliosis enforces that poor connective tissue development as possible etiological factor. Despite the coexistence of pectus carinatum and scoliosis has attracted the attention of some researchers, the association between pectus carinatum and the other spinal deformities has not been studied comprehensively. The frequency of spinal deformity in patients with pectus carinatum and the mutual relationships of their subtypes are needed to be studied to determine the epidemiological character of the combined deformity and to plan patient evaluation and management. Our study aimed to investigate the association, define the incidence and evaluate the characteristics between different types of spinal deformities and Pectus carinatum. Materials and Methods. Radiological and physical examinations were performed for 117 pectus carinatum patients in Marmara university hospital/Turkey in the years between 2006 and 2013. The incidence of spinal deformity was calculated. Spinal deformities were classified as scoliosis, kyphosis, kyphoscoliosis, and spinal asymmetry, whereas pectus carinatum were subdivided into symmetric and asymmetric subgroups. The relationship between spinal deformities and the symmetrical-asymmetric subtype of pectus excavatum was statistically analyzed, Pearson chi-square test was used to compare the association of qualitative data. The significance level was accepted as p <0.05. Lastly, the angular values of the deformities of scoliosis and kyphosis patients were measured using the Cobb method. In this way, the magnitude of the deformity was given as a numerical value. Results. Spinal deformity was detected in 23 (17 symmetrical PE and 6 asymmetrical PE) of 117 pectus excavatum patients. Scoliosis and kyphosis were seen equally in symmetrical pectus carinatum, whereas scoliosis was seen in 33.3% and kyphosis in 50% in asymmetric pectus carinatum patients, respectively. However, there were no statistically significant differences in the distribution of scoliosis and kyphosis in patients with symmetrical and asymmetrical PE. Idiopathic scoliosis constituted the most common scoliosis group. Congenital kyphosis was not found in any kyphosis patient. The average Cobb angle of scoliosis patients was 32°, and the mean T2-T12 kyphosis angle of these patients was 55.5°, while the average kyphosis angle of those with kyphosis deformity was 71°. Conclusions. Patients with Pectus carinatum have a higher incidence of spinal deformities than the normal population. Such high concomitant incidence should be taken under consideration in evaluating and treating patients presenting with either deformity

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

Summary of Background Data: The craniofacial malformations described by Goldenhar can be associated with congenital anomalies of the vertebrae. This non-random association of abnormalities represents unilateral errors in the morphogenesis of the spine, as well as the first and second branchial arches. Purpose of the study: The aim of the present study was to determine the prevalence of Goldenhar related conditions in patients with congenital deformities of the spine and to describe the types of vertebral abnormalities and the necessity for treatment. Material-Methods: We performed a retrospective study of 668 consecutive patients with congenital deformities of the spine. The medical records and spinal radiographs were reviewed and patients with a Goldenhar associated condition were identified. The vertebral anomalies causing the spine deformity were detected on antero-posterior and lateral spine radiographs. The type and site of the craniofacial abnormalities, as well as other musculoskeletal deformities and systemic anomalies were recorded. Results: Fourteen patients had Goldenhar associated conditions (7 males and 7 females). A thoracic scoliosis was the most common type of deformity occurring in ten patients (71.5%). Eight of these patients had an isolated hemivertebra and the remaining two had a unilateral unsegmented bar with contralateral hemivertebra at the same level. There was only one patient with a lumbar scoliosis and this was due to a hemivertebra. The side of the vertebral anomaly correlated with that of the hemifacial microsomia in five of the eleven patients who had a scoliosis or kyphoscoliosis. A thoracolumbar kyphosis occurred in four patients; two had posterior hemivertebrae, one had wedge vertebrae, and the remaining patient had an anterior unsegmented bar. A thoracolum-bar kyphoscoliosis occurred in only one patient and was due to a posterolateral quadrant vertebra. Klippel-Feil syndrome occurred in six patients (42.8%). Eight patients (57%) underwent surgical treatment at a mean age of 9.8 years (range: 2.9–19). Four patients had a combined anterior-posterior spine arthrodesis. The remaining four patients had a posterior spinal arthrodesis. Conclusions. The prevalence of Goldenhar associated conditions in patients with congenital deformities of the spine was 2%. Failures of vertebral segmentation were the most frequent abnormality in the cervical spine, whereas failures of vertebral formation most commonly occurred in the thoracic or thoracolumbar spine

Introduction and Purpose of Study. Osteogenesis imperfecta (OI) is a bone metabolic disorder that results in multiple fractures and deformities in children. The management of these patients should be in highly specialised units were multi-disciplinary management is mandatory. The aims of this study were twofold: 1. To determine the incidence and pattern of fractures in this population. 2. To determine the type, outcomes and complications of surgical treatment in the same population. Methods. A retrospective audit of patients treated for OI at a tertiary academic Hospital, from January 2002 to December 2011 was done. Results. Fifty three patients with OI were seen in the period under review. The patients came from six South African provinces including two other African countries. The male to female ratio was 1:1. The majority of patients were classified as type III and type IV, 19 (36%) and 14 (26%) respectively. Twelve patients (23%) had a first degree relative with OI. All patients received bisphosphonate therapy intravenously except two who were on oral medication. Seventeen patients (33%) had associated kyphoscoliosis – none were treated surgically. The most common long bone fractures were of the midshaft femur (61 fractures) and tibia (35 fractures). Seventeen patients (32%) received intramedullary rodding of either femur or tibia. Surgery had to be repeated due to rod migration in nine long bones (29%). The most common complication of surgery was rod migration and peri-implant fracture. Conclusion. Long bone fractures of the femur and tibia were most common cause of morbidity. Intramedullary rodding is a safe and effective means of long bone fracture management in patients with OI

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

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

To describe complications and reoperation rates associated with degenerative spinal deformity surgery. A retrospective review of prospectively collected data from a single spinal surgeon in the United Kingdom. A total of 107 patients who underwent surgery, of 5 or more levels, for primary degenerative kyphoscoliosis between 2006 and 2012 were identified. Clinical notes were reviewed and post-operative complications, reoperation rates, length of follow up and mortality were analysed. A total of 107 patients, average aged 66.5 years (range 52 – 85), with 80% women. 105 patients underwent posterior surgery, two patients required both anterior and posterior surgery. The average number of instrumented levels was 8.3; 10% 5 levels, 15% 6 levels, 11% 7 levels, 14% 8 levels, 15% 9 levels and 35% had fusions of 10 levels and above. 58% included fixation to sacrum or pelvis. 93% had a decompression performed and 30% had an osteotomy. There were 40 complications recorded within the follow-up period. Infection occurred in 7 patients (6.5%). All were successfully managed with debridement, antibiotic therapy and retention of implants. There were 4 dural tears (3.7%). One patient developed a post-operative DVT (0.9%). No patients sustained cord level deficits. Prevalence of mechanical complications requiring re-operation was 26% (28 patients). 5 patients (4.7) required revision surgery for symptomatic pseudarthrosis, 7 patients (6.5%) underwent revision fixation for metal work failure (broken rods/screw pull-out) and 16 patients (14.9%) underwent revision surgery to extend fixation proximally or distally due to adjacent segment disease (symptomatic proximal junction kyphosis 4.7%; osteoporotic fracture 3.7% and junctional/nerve root pain 6.5%). Overall reoperation rate was 32.5% at an average of 1.9 years following primary surgery (range 1 week–6 years). 37% patients remain on regular outpatient review (average 3.8 years following first surgery; range 2–6 years). 52% have been discharged after a mean follow-up of 2.3 years. 11 patients had died since their surgery (10.2%) at an average 4.1 years following their spinal surgery (range 1 –5.9 years). Overall complication rate was 37.3%. 32.5% of patients were re-operated for infective or mechanical complications. 52% of patients had been discharged at an average of 2.3 years following their surgery. 10.2% of patients had died within 6 years of surgery

Purpose. compare the radiological results in sagittal balance correction obtained with pedicle subtraction osteotomy (PSO) versus anterior-posterior osteotomy (APO) by double approach in adults. Material and Methods. between January of 2001 and July of 2009, fifty-eight vertebral osteotomies were carried out in fifty-six patients: 9 Smith-Petersen osteotomy (SPO), one vertebral resection osteotomy (VRO), 30 anterior-posterior osteotomies (APO) and 18 pedicle subtraction osteotomies (PSO), being the lasts two groups the sample studied (48 osteotomies). The mean age of the patients was 56.3 years (17–72). Initial diagnose was: 28 posttraumathic kyphosis, 7 postsurgical kyphosis, 7 adult degenerative disease, 4 ankylosing spondylitis and 2 congenital kyphoscoliosis. We evaluated the preoperative standing radiographs, the postoperative and at final follow-up by digital measurements with iPACS system viewer (© Real Time Image, USA, 2001). The mean follow-up was 54 months (6–98), and complications were analized. Results. The group APO had a mean preoperative thoracic kyphosis of 67 °, a mean lumbar lordosis of −42° and a mean sagital balance of 8.6°. The group PSO had a mean preoperative thoracic kyphosis of 41°, a mean lumbar lordosis of −22° and a mean sagital balance of 12.3°. The mean correction in the APO group was 29° in its thoracic kyphosis, 8° of lumbar lordosis and 6.5° in its sagital balance. The mean correction in the PSO group was 12° of the thoracic kyphosis, 25 in the lumbar lordosis and 8.4 cm in the sagital balance. The local correction obtained at the osteotomy level was 28° in the APO group and 25.3° in the PSO group. There were no statistically significant differences in the percentage of correction between both groups (p>0.05). In terms of complications, PSO group had lower complication rate (26.6%) comparing to ODV group (44.5%). Conclusions. APO and PSO are useful techniques to correct the global sagital balance in patients with a disturbance of the sagittal profile. The correction obtained with the PSO is similar to obtained with the APO. Patients undergoing an OSP had a lower complication rate than patients undergoing APO

Purpose of the study: Diastematomyelia is a rare spinal cord malformation defined as the presence of two separate spinal cords separated or not by an osseous, cartilaginous, or fibrous septum. Spinal malformations are almost always associated, raising difficult therapeutic challenges. Material and method: We report three cases of congenital kyphoscoliosis associated with diastematomyelia in three girls aged 12, 14 and 15 years. The diastematomyelia was dorsal in one case, thoracolumbar in one and lumbar in the third. For all three patients, the indication for surgery was progression of the scoliosis with development of neurological signs of recent progressive aggravation. Preoperative distraction with a plaster cast was pursued for several months prior to posterior instrumentation. No attempt was made to correct the cord malformation nor achieve major correction of the spinal malformation. The instrumentation bridged the thoracolumbar scoliosis in one case and stopped above the malformation in the two others. Results: The postoperative period was uneventful. There were no neurological complications. Preoperative neurological signs improved after surgery. Control radiographs showed an improvement in the deformity. At mean 6 years follow-up, these patients were not bothered in their everyday life. They had stable deformities which a globally balanced trunk. There were no signs of neurological evolution. Discussion: The therapeutic strategy for diastematomyelia remains a subject of debate. For some authors, the spinal cord should be released systematically which for the majority, this is not necessary except if spinal distraction is planned or if there is a neurological problem. If there is an indication for spinal cord release, any spurs must be removed followed by the necessary dura mater plasty. In our three patients, and in agreement with the neurosurgery team, there was no need for neurosurgical release. The recent development of neurological deficits was explained by the important kyphosis rather by the intramedullary anomaly. Our therapeutic strategy thus focused on treatment of the scoliosis. This enabled us to stabilise the spine, protecting these patients from worsening neurological involvement and enabling good functional outcome. The zone of the malformation was not instrumented in all cases because the posterior arcs were deformed, but also to avoid compromising any future neurosurgical intervention

Study design. Prospective clinical and radiological analysis of children with complex cervical deformities for the safety of cervical pedicle screw insertion. Objectives. To analyse the possibility, safety and efficacy of cervical pedicle screw insertion in complex pediatric cervical deformities, where conventional stabilisation techniques would not have provided rigid fixation. Summary of Background Data. Although the usage of cervical pedicle screws (CPS) in adults has become established, the feasibility and safety of its application in children has not been described previously in the literature. Methods. Sixteen children of mean age 9.7 ± 2.6 years (range: 3 - 13) requiring spinal stabilization for cranio-vertebral junction anomalies (n=10), cervico-thoracic kyphosis/ kyphoscoliosis (n=5) and cervical tumor excision (n=1) formed the study group. Feasibility of CPS insertion was assessed by computerised tomography images. Standard 3.0 mm titanium pedicle screws were inserted using intraoperative Iso-C C arm based 3 D computer navigation and the containment was post operatively evaluated with CT scan. Results. Based on preoperative CT imaging, 55 pedicles were selected for screw fixation. Intra operatively CPS was successfully inserted at 51 levels and at four sclerosed pedicles (7.3%), screws could not be inserted. At 42 levels, the screws were inserted in the classical description of pedicle screw application and in nine deformed vertebra, the screws were inserted in a non-classical fashion, taking purchase in the three columns of the cervical vertebra. Forty five (88.3%) screws were fully contained, six (11.7 %) had a non-critical breach and none had a critical breach. No perioperative complications related to pedicle screw insertion were noted. Conclusion. Safe insertion of cervical pedicle screws is possible in children. Iso-C navigation provides real time virtual imaging and improves the safety and accuracy of successful pedicle fixation even in altered vertebral anatomy. Pedicle width morphometrics do not restrict screw insertion

Introduction. Pedicle Subtraction osteotomy (PRO) in correction of severe spinal deformities is well established. Prospective analysis of its efficacy in complex spinal deformities is sparse in literature. Aims and objectives. To assess the role of PRO in correction of uniplanar and multiplanar spinal deformity and to assess the role of revision PRO in failed corrections. Material and methods. 50 patients were operated between 1996-2007 and followed up for 2 years (2-6). 27 had uniplanar kyphosis (60-128 degrees) and kyphoscoliosis was seen in 10. Failed corrections were seen in 11 uniplanar and 2 multiplanar deformities. The average pre-operative kyphosis and sagittal balance was 78.7 degrees and 22 mm (7-30) respectively. Scoliotic deformity ranged from 97-138 (average 108 degrees) and the coronal imbalance from 10-55 (average 24mm). Deformity distribution was upper dorsal 5, mid dorsal 22, dorso-lumbar 18 and lumbar 5. A single posterior approach sufficed in 47 cases while 3 required an anterior approach for reconstruction. 13 patients had pre-operative neurological deficit (bedridden 10, ambulatory 3). The average surgical time required was 300 minutes and blood loss was 800cc. The anterior defect reconstructed averaged 16.5mm (5-28). Results. Pulmonary complications occurred in 8 (21%), (embolism 1, pneumonia 2, hypoxia 5). Wound infection required debridement in 3 (8%). Failed corrections were seen in 10 (3 out of 37 in our series, 8%) due to failure of construct 2, severe disease 2, infection (active 2, quiescent 4). Neurological deterioration occurred in 1(2%), medial pedicle wall perforation. 12 patients regained ambulation (independent 7, support 5). Post-operative kyphosis and sagittal balance was 36.5 (10-108) and 10mm (5-20) respectively. Average correction was: sagittal 46.4%, coronal 37.5% and revisions 58%. The correction of kyphosis and sagittal balance was statistically comparable between primary and revision cases (p >0.05). Conclusions. PRO offers an excellent single stage decompression and controlled correction of kyphosis

Abstract: It is known that the treatment of intra or extraspinal paediatric tumours with surgery and radiotherapy or radiotherapy alone can lead to the onset of progressive spinal deformity the management of which can be extremely challenging. We review our series of patients who have developed a spinal deformity in these circumstances. Methods: A review of all patients seen between 1996 and 2007 in the spinal department who have developed a significant spinal deformity following treatment for an intra or extra spinal tumour. Results: 14 patients were identified. The age of presentation to the spinal service was between 2 years 6 months and 15 years 3 months. The underlying diagnoses were Wilms Tumour treated with surgery and radiotherapy in 3, 1 extraspinal sarcoma treated with surgical resection, radiotherapy and chemotherapy, 1 extraspinal neuro-blastoma treated with surgery, radiotherapy, chemotherapy and stem cell rescue and 9 intra spinal tumours (PNET, astrocytoma, ganglioneuroblastoma and der-moid) all managed with resection with or without radiotherapy. The spinal deformities that have developed were thoracic kyphoscoliosis, thoracolumbar kyphosis and lumber hyperlordosis. The spinal management of these deformities has been conservative in 12 with regular assessment to allow intervention if indicated. 2 patients have undergone surgery, a vascularised fibular strut graft and anterior instrumentation in 1 and a non vascularised rib graft in 1 for progressive deformity felt to lead to neurological dysfunction or lung hypoplasia. The cases managed operatively were complicated by poor posterior soft tissues following previous surgery and radiotherapy requiring an anterior approach. In all patients who were treated with radiotherapy platyspondyly was always seen in the vertebral bodies anteriorly and this corresponded to the apex of the deformity. Conclusion: In all children who undergo spinal surgery and or radiotherapy for paediatric tumours there must be ongoing surveillance for the development of a spinal deformity. We feel that this is as much a result of anterior growth arrest secondary to radiotherapy as to posterior laminectomy for intra canal tumour excision. The surgical management of this problem is complex and may require innovative solutions

Introduction and Aims: Although PD has characteristic findings and stages of progression, neither the nature of operative spine problems occurring in patients with PD, nor their peri-operative complications, have been presented previously. Method: In this retrospective observational case series, we describe the nature of operative spine problems in patients with Parkinson’s disease (PD), and evaluate the incidence and types of peri-operative complications in spine surgery for patients with PD. A retrospective chart review was performed on 24 consecutive PD patients undergoing spine surgery in a six-year span at one institution. Nineteen were first surgeries, and five were first seen as failed back syndrome from surgery done elsewhere. Results: The mean patient age was 69 years. Spinal stenosis (10 patients) and cervical myelopathy (4 patients) were the predominant pre-operative diagnoses for the primary surgeries, with three patients operated upon for kyphoscoliosis (16%) and two for other conditions. Minor or transient complications were common, including delirium, arrhythmia, deep vein thrombosis, and dural tear; there were also two significant PD-specific complications, including aspiration pneumonia and global motor paresis post-operatively. Four of 19 primary cases were revised for recurrent stenosis (two patients) or pseudarthrosis (two patients). The overall number of poor patient-reported outcomes was only two of 14 primary cases (five patients had inadequate outcome measures) and zero of seven revisions (two had inadequate outcome measures), at a mean follow-up of 19 months. Conclusion: Symptoms and functional deficits of spinal disease were often masked by PD, posing diagnostic difficulty. However, outcomes and complications of spine surgery were similar to those of non-PD patients at the same institution. Spine symptoms improved concomitantly with successful surgery unless the PD progressed or significant complications ensued

Study Design: Retrospective study with clinical and radiological evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebae (HV). 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 kyphosis in children below the age of 3. We discuss the management of patients presenting with neurological compromise. Summary of background data: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique. Materials and Methods: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. 13 patients had a single posterolateral HV and 2 patients a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range 8–33). Mean follow-up period was 6.8 years. 13 HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine. Results: The average operating time was procedure was 150 minutes (range, 130 to 210 minutes). The average blood loss 180 mL (range, 100 to 330 mL), equivalent to a mean external blood volume loss of 15% (range, 11 to 24%). Preoperative segmental Cobb angle averaging 34 º at last follow up. Compensatory coronal cranial and caudal curves corrected by 50%. The angle of segmental kyphosis averaged 39º (range, 20º to 80º) before surgery and 21 º (range, 11º to 40º) at last follow up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last followup. One case with initial kyphosis of 80 º continued to progress and required revision anterior and posterior surgery. There were no neurologic complications. Key points:. In progressive congenital kyphoisis, early diagnosis and aggressive surgical treatment are mandatory for a successful result. Early treatment minimizes the risks of surgery. Anatomical and physiological pitfalls in the treatment of congenital kyphosis are discussed. Anterior instrumented fusion of congenital kyphosis provides sagital and coronal correction in very young children with low risk of complications

Purpose: We reviewed our pre-puberty patients whose scoliosis or kyphoscoliosis involved a hemivertebra. The purpose of our work was to evaluate the surgical technique used and evaluate spinal static as well as functional outcome at skeletal maturity. Material and methods: This retrospective analysis included 21 patients who underwent surgery before the age of 10 years and were followed to skeletal maturity. We excluded children with a multiple malformation syndrome or multiple vertebral malformations. The type and localisation of the hemivertebra was noted. Deformation, transversal balance, and radiographic measures were recorded preoperatively. Elements contributing to the indication for surgery, the type of procedure, and complications were also recorded. Events recorded during growth were the clinical course, complementary treatments, and possible surgical revision. Functional and aesthetic outcome was assessed at last follow-up. Spinal deviation was measured and compared with the preoperative angles. Results: Twenty-one children (13 girls, 8 boys), mean age three years ten months (range 10 months – 10 years) met the inclusion criteria. These children had a hemivertebra of the thoracic spine (n=9), the thoracolumbar junction (n=4), the lumbar spine (n=4), or the lumbosacral region (n=4). Surgery was indicated to arrest clinical and radiographic degradation. Several surgical procedures were used: fusion without resection for thoracic vertebrae and resection associated with arthrodesis or epiphysiodesis for other localisations. There were five complications: neurological (n=2), infectious (n=2) and disassembly (n=1). The clinical and radiographic course led to revision in ten children, including two who required a new operation. At mean follow-up of 14 years (9–23 years), the functional outcome was good in 19 patients, poor in one patient with a lumbosacral hemivertebra and in another with a thoracic hemivertebra. The aesthetic result was good in 16 patients. Five of the nine patients with a thoracic hemivertebra remained unsatisfied with the outcome. Mean curvature correction ranged from 26% at the thoracic level to 50% at the thoracolumbar and lumbosacral levels and 75% at the lumbar level. Discussion: The long follow-up of this series is exceptional. Treatment of evolving spinal malformations is a difficult challenge. Early surgery does not guarantee the final outcome and 50% of patients have to be reoperated at the end of growth. The good long-term functional and aesthetic outcome is however encouraging, particularly when hemivertebrectomy can be performed

Study Design: A retrospective analysis of patients with spinal disorders using Magnetic Resonance Imaging (MRI) results. Objective: To review the clinical and MRI results of patients with various scoliotic deformities. Background: Insufficient reports exist regarding the MRI’s of scoliotic deformities. MRI’s can offer vital information in the diagnosis of various types of scoliosis and their concomitant disorders. Methods: MRI reults of a total of 277 patients with various types of scoliosis/kyphoscoliosis were reviewed. All patients met the cobb angle criteria: > or = 20 degrees. 65 (23.46%) patients were male and 212 (76.53%) were female. 224 (80.86%) patients received conservative treatment and 53 (19.14%) underwent surgical treatments. 107 (38.62%) patients had adolescent idiopathic scoliosis, mean age: 13.7 (7–18) years, 76 (27.43%) adult idiopathic, mean age: 29.53 (19–79) years, 48 (17.32%) congenital, mean age: 12.6, (1–46) years, 29 (10.46%) neuromuscular, mean age: 12.86 (2–30) years, 15 (5.41%) syndromic, mean age: 13.6 (1–29) years, 2 (0.72%) tumor related, mean age:10.5 (8–13) years. Results: MRI results revealed the spinal cord of 169 (61.01%) patients as normal: no spinal cord anomalies, tumors, or congenital problems. Of the remaining patients 108 (38.98%) the following irregularities were diagnosed: 39 (36.11%) syringohydromyeli in various spinal locations, 29 (26.85%) butterfly vertebrae, 19 tethered cord (17.59%), 10 (9.2%) split cord, 10 diastometamyelia, 10 cleft vertebrae, 14 (12.96%) myelomeningocele, 7 (6.48%) grade one spondilolisthezis, 5 (4.62%) caudal regression syndromes, 6 (5.55%) vertebra partial fusion, 4 (3.7%) cranio-cervical problems, 4 cerebellar tonsillar ectopia, 3 (2.77%) block vertebra, 3 chiari typ2 II, 3 TIS, 2 (1.85%) tumors on the spinal column, 2 neurofibromatosis, 2 introdural lipoma, 2 myelomalacia of the spinal cord, 2 spinal cord injuries, 1 (0.92%) arachnoid cyst, 1 neuroanteric, 1 spina bifida, 1 scheuermann, 1 vertebral artery hypoplasia, 1 sacral dermal sinus, 1 cervical rib, 1 interpedicullar cyst, 1 high scapula, 1 sphenoid sinus retention cyst, 1 paravertebral cyst, 1 Schmorl’s node, 1 Tarlow cyst and 1 intercranial pineal cyst. Conclusion: Our study revealed how MRI analysis can lead to the accurate diagnosis of scoliotic deformities. In many cases tumors, neuromuscular pathology and syndromic conditions can be misdiagnosed as scoliotic. Careful MRI review can offer vital information for diagnosis and help determine the classification of scoliosis and subsequent treatment