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

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

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

Introduction: Congenital kyphosis is an uncommon deformity caused by failure of the vertebral bodies to form and/or segment. It is treated surgically. Our purpose is to confirm whether the treatment protocols established years ago are still valid and to assess the benefits of improved implants. Materials and methods: Between 1985 and 2003, 24 patients underwent surgery in La Paz Hospital. They were classified into three groups: < 5 years (8 p), 6–12 (6 p) and > 13 (10 p). The minimum follow-up was 2 years (2–14). The procedures were posterior or circumferential spinal fusion, with or without instrumentation, and corrective osteotomy. Complications were evaluated radiographically. Results: In the first group posterior spinal fusion was performed in six patients and circumferential in two, with a correction rate of 55%. In the second group instrumented circumferential spinal fusion was performed in three cases and instrumented posterior in three (30% correction). In the third group eight of the ten patients underwent instrumented anteroposterior spinal fusion (osteotomy in five) and the correction rate was 45%. Complications: 3 pseudoarthrosis, 1 DVT, 2 infections and 3 failed implants. Conclusions: Congenital kyphosis can be controlled at any age, although early surgery is best. Gradual correction occurs after poster spinal fusion in children and instrumentation prevents revision of the fusion and prolonged immobilisation. Persons over the age of 5 with type I kyphosis usually require circumferential spinal fusion

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

Objective: To assess if a minithoracotomy gives sufficient access to undertake satisfactory anterior release and fusion of the thoracic spine. Design: A prospective collection of data in patients undergoing anterior spinal surgery. Subjects and Methods: Ten patients, nine with adolescent idiopathic scoliosis (AIS) and one with congenital thoracolumbar kyphosis underwent anterior release and fusion through a minithoracotomy. A minithoracotomy being defined as a thoracotomy through an incision of ≤ 6cm. The female to male ratio was 3: 2 with an average age at the time of surgery of 13.5 years (10–15) in the scoliosis group. The patient with congenital kyphosis was operated on at 24 years of age. The mean standing pre-operative Cobb’s angle in the AIS group was 78.4° (60–110°), and this was reduced to a mean of 64° (45–85°) on bending films, with a flexibility of 18.4% (2.7–40%). The pre-operative kyphosis angle in the kyphosis patient was 60°. The thoracotomy incisions were measured with a sterile measuring tape on the completion of closure. Results: All anterior surgery was satisfactorily achieved through a minithoracotomy with mean incision length of 6.5 cm (5.5–7). The average time taken for thoracotomy and procedure was 99 minutes (40–120) and an estimated blood loss of 116mls (50–250). Satisfactory correction was achieved in all patients with the mean improvement of the Cobb’s angle of 56% and post-operative Cobb’s angle of 34° (18–52). The post-operative kyphosis angle in the thoracolumbar kyphosis patient was 45°. The mean post-operative stay in the intensive care unit was 1.2 days (1–2); the duration of chest drain 1.5 days (1–2); blood transfusion requirement 2.9 units (1–6) and the post-operative stay on the ward 7 days (6–8). Two complications were seen comprising of minor chest infection and superficial infection of the chest drain wound requiring a course of oral antibiotics. Conclusion: A minithoracotomy provides satisfactory access to achieve anterior release as demonstrated by satisfactory correction achieved with improved cosmesis, acceptable operation time and blood loss and with minimal complications

Treatment of congenital kyphosis with severe angular dysplastic spine in children with myelomeningocele (MMC) is one of the most difficult spinal procedures. Most of the surgeons support kyphectomy with long segmental spinal instrumentation and postoperative immobilization by thoracolumbosacral orthosis. Several spinal deformities are seen frequently in patients who have MMC. The deformity may be congenital or paralytic. Congenital lumbar kyphosis is less common, but most difficult in patients with MMC, occurring in 10–20% of patients. Most curves are congenital and rigid, often more than 80° at birth, and rapidly progresses. With progression of kyphotic deformity, patients experience recurrent skin breakdown over the apex of the kyphos; impaired sitting balance; the necessity of using their hands for support; collapsing spine and decreasing of lumbar height reduce the capacity of the abdominal cavity and resulting in reduced respiratory capacity and malnutrition. The poor posture and short abdomen make it difficult to manage the patients’ urological needs. A severe deformity raises difficulties in social and psychological development. Non-operative treatment with spinal orthoses may provide only temporary correction of a kyphotic deformity, but does not prevent progression and skin breakdown. The goal of surgical treatment is correction of spinal deformity by long segmental instrumentation and achievement of a solid spine fusion in order to allow a balanced sitting position and to prevent complications. From 1983 to 2001, 6 patients with thoracic level myelomeningocele and severe kyphotic deformity were referred for surgical correction. There were 5 males and 1 female patients with average age at the time of surgery of 8.3 years (range 4.3–13 years). All patients suffered from severe kyphosis, range 90° to 130°, average – 108°. All of them underwent posterior ligation of spinal cord during resection of lordotic segment of the kyphos, and segmental spinal fixation of the deformity from the thoracic spine to the sacrum. In all cases following the resection of the vertebrae it was possible to correct the deformity. All patients were available for follow-up with range of 6–216 months, average 85 months. All of them were satisfied with the surgical outcome and presented in their final clinical examination with balanced and comfortable sitting, without soft tissue complications. In all cases a significant correction of the deformity was achieved (15°–30°) and enabled comfortable and stable sitting. Two patients suffered post-operative complications, one from surgical wound infection which required surgical debridement followed by soft tissue covering, and the other suffered from distal migration of the rod which was shortened later on. Discussion: Kyphotic deformity in a patient who has MMC is a challenge for the orthopaedic surgeon and requires major surgical intervention. Resection of the kyphos with posterior instrumentation and fusion may solve patient’s functional problems

Introduction: Since 1989 vertebral resection with modified Luque fixation has been the procedure of choice for correction of myelomeningocele kyphotic deformity at this institution. The purpose of this study was to evaluate long-term results with this technique. Treatment or congenital kyphosis in myelomeningocele is a difficult problem. Current thinking supports kyphectomy and post-operative internal fixation. The majority of authors agree that kyphotic deformity in myelomeningocele should be treated with vertebral resection. There is less uniform consensus as to postoperative fixation. Literature reports appear to support fixation with modified segmental instrumentation. Methods and Results: 16 patients, followed for an average of 57.2 months (36–94 months), underwent vertebral resection from the proximal aspect of the apical vertebra cephalad into the compensatory lordotic curve. Fixation was segmental instrumentation wired to the thoracic spine and anterior to the sacrum. The average blood loss was 1121 cc (450–2580 cc.). Pre-operative kyphotic deformity averaged 111° (75–157°), postoperative 15° (−18° –36°) and latest follow-up of 20° (−17° –83°), with loss of correction of 6° (0–27°). Post-operative immobilisation was with a TLSO for 18 months. Complications occurred in eight of the 16 patients: (1) transient headache (2), superficial wound breakdown (2), supracondylar femur fractures (2), and one late infection secondary to skin breakdown necessitating early rod removal and some loss of correction. Conclusions: Kyphectomy is an excellent method of correcting rigid kyphotic deformity in the myelodys-plastic patient. Segmental spinal instrumentation provides three distinct advantages: rigidity of the construct, greater correction of the deformity and low-profile instrumentation

Purpose: To determine the results and safety of patients undergoing|spinal cord level (SCL) pedicle subtraction osteotomy (PSO) for thetreatment of thoracic kyphosis. Methods: Retrospective chart and radiographic review of 25 patients with severe thoracic kyphosis. Results: The underlying diagnoses were: tumour (8), Scheuermann’s Kyphosis (4), degenerative/osteoporosis (3), fracture (3), inflammatory|(2), neurofibromatosis (2), congenital kyphosis (1), tuberculosis (1), and infected tumour (1). The osteotomy was combined with a lumbar PSO in|five patients. Three patients were treated with double thoracic|osteotomies. Two PSOs were extended transdiscally to debride the|infected disc. The mean focal PSO correction was 33.6° (range 9°–73°). The overall thoracic kyphosis measured from T5 to T12 improved from a|mean of 58.3° preoperatively to 37.1° postoperatively. Estimated blood|loss ranged from 400cc to 12500cc. All patients presenting with spinal|cord dysfunction neurologically improved postoperatively. There were 2 major neurological complications. One patient developed postoperative | progressive paraplegia following a prolonged period of intra- and | postoperative severe hypotension and coagulopathy. The other developed a| pseudoarthrosis five months postoperatively and suffered an incomplete|spinal cord injury during the subsequent revision. Other complications included: T3 radicular pain (1) -resolved; dural tears (2); respiratory failure -prolonged ICU admission(1); fractures proximal to the thoracic (2) and distal to lumbar (1) instrumentation; incomplete corrections of the sagittal alignment despite double osteotomies (2); wound breakdown associated with preoperative radiation (1).|. Conclusions: SCL-PSO is a feasible option for severe thoracic kyphosis. This procedure eliminates the need for anterior surgery; however, it does not reduce the potential for significant morbidity

Introduction. The aim of the treatment of children with early onset scoliosis is controlling growth of the spine. Whatever the etiology, early progressive deformations require multiple stages of surgery usually performed every 6–12 months. One have to be reckoned with complications requiring additional surgical intervention. Objective. The aim of the study is to present a new method of surgical treatment of early onset scoliosis involving the implantation of specially constructed implants to allow three dimensional correction of spinal deformity with a preserved capacity to continue the growth of spine without distraction staged operations followed by final spondylodesis in mature spine. Material. The clinical material consists of homogeneous group of patients: 8 girls and a one boy aged 6 to 14 years (mean age = 9 years). The estimated group four children had a single-curve, four children had a double-curve, while one child was affected by congenital kyphosis. The follow up ranged from 2 to 17 months (mean = 13.5 months). Method. Efficacy of spinal deformity correction using a “growing implants” was estimated by Cobb andgle measurement of the curvature 1/before the operation, 2/after surgery and 3/follow up. Results. During surgery, all patients obtained a large correction of curvature ranging from 50% to 100% (on average −70%). The degree of correction was directly dependent on the size of the initial deformation of 62 to 120 ° (average 77 °). During the entire period of observation in four children we have not identified the loss of correction or fits within the limits of measurement error. In one child thirty degree-loss correction stemmed from too selective implantation of the implants. During additional surgery the stabilization was extended to the extra two motor segments witch resulting in full correction. In one patient, due to rapid growth, rods were needed to be replaced for longer, because of the risk of pulling out from the lower screws. In one case we observed further correction during follow up. Conclusions. Using the method we obtained a very good correction in the first stage of treatment. Maintenance of correction does not require any intermediate staged operating procedures. Patients do not require corrective brace. Using “growing implants” in the early onset scoliosis one avoid complications peculiar to current growth-sparing procedures. These patients would have had 15 lengthening procedures after their initial correction if treated by conventional growing rod methods