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Purpose: Intracanal rib head penetration is a well-known entity in dystrophic scoliotic curves in neurofibromatosis type 1. There is potential for spinal cord injury if this is not recognised and managed appropriately. No current CT-based classification system is currently in use to quantify rib head penetration. This article aims to propose and evaluate a novel CT-based classification for rib head penetration primarily for neurofibromatosis but which can also be utilised in other conditions of rib head penetration., Materials and Methods: The grading was developed as four grades: normal rib head (RH) position-Grade 0, subluxed extracanal RH position-Grade 1, RH at pedicle-Grade 2, intracanal RH-Grade 3. Grade 3 was further classified depending on the head position in the canal divided into thirds. Rib head penetration into proximal third (from ipsilateral side)-Grade 3A, into the middle third-Grade 3B and into the distal third-Grade 3C. Seventy-five axial CT images of Neurofibromatosis Type 1 patients in the paediatric age group were reviewed by a radiologist and a spinal surgeon independently to assess interobserver and intraobserver agreement of the novel CT classification. Agreement analysis was performed using the weighted Kappa statistic., Results: There was substantial interobserver correlation with mean Kappa score (k = 0.8, 95% CI 0.7-0.9) and near perfect intraobserver Kappa of 1.0 (95% CI 0.9-1.0) and 0.9 (95% CI 0.9-1.0) for the two readers., Conclusion: The novel CT-based classification quantifies rib head penetration which aids in management planning., (© 2021. Scoliosis Research Society.)

Background: Final fusion is thought to be the end point for patients with early onset scoliosis following treatment with the use of growing rods. But is it? The purpose of this study was to determine the incidence and cause of any reoperation after final fusion., Methods: A multicenter database of patients with early onset scoliosis was retrospectively analyzed to identify patients treated with growing rods with a minimum of 2 years of follow-up after final fusion. All reoperations were recorded. Reoperation was defined as a return to the operating room for any complication related to the final fusion surgery or etiology of the spinal deformity., Results: One hundred (84%) of 119 patients met the inclusion criteria: for 38 of the patients, the etiology of scoliosis was neuromuscular; for 31, syndromic; for 22, idiopathic; and for 9, congenital. The mean age at final fusion was 12.2 years (range, 8.5 to 18.7 years). The mean follow-up after final fusion was 4.3 years (range, 2 to 11.2 years). Twenty (20%) of the patients had 30 complications requiring reoperation (57 procedures). There was a mean of 1.5 complications per patient after final fusion. Eight patients with neuromuscular scoliosis, 8 with syndromic, 4 with idiopathic, and no patient with congenital scoliosis required reoperation. Nine (9%) of the patients experienced infection (33 reoperation procedures); 6 (6%) had instrumentation failure (8 procedures); 5 (5%) had painful or prominent instrumentation (6 procedures); 3 (3%) each had coronal deformity (3 procedures), pseudarthrosis (3 procedures), or sagittal deformity (3 procedures); and 1 (1%) had progressive crankshaft chest wall deformity requiring a thoracoplasty (1 procedure)., Conclusions: A higher-than-anticipated percentage of patients treated with growing rods required unplanned reoperation following final fusion. Long-term follow-up after final fusion is necessary to determine true final results. Patients and parents need to be counseled regarding the possibility of further surgery after final fusion., Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Deep surgical site infection may change the course of growing-rod treatment of early-onset scoliosis. Our goal was to assess the effect of this complication on subsequent treatment., Methods: A multicenter international database was retrospectively reviewed; 379 patients treated with growing-rod surgery and followed for a minimum of two years were identified. Deep surgical site infection was defined as any infection requiring surgical intervention., Results: Forty-two patients (11.1%; twenty-five males and seventeen females) developed at least one deep surgical site infection. The mean age at the initial growing-rod surgery was 6.3 years (range, 0.6 to 13.2 years) and the mean duration of follow-up was 5.3 years (range, 2.2 to 14.3 years). The mean interval between the initial surgery and the first deep surgical site infection was 2.8 years (range, 0.02 to 7.9 years). Ten (2.6%) of the 379 patients developed deep surgical site infection before the first lengthening. Twenty-nine patients (7.7%) developed the infection during the course of the lengthening procedures, and three patients (0.8%) developed it after final fusion surgery. Thirty (13.6%) of 221 patients with stainless-steel implants had at least one deep surgical site infection compared with twelve (8%) of 150 patients with titanium implants (p < 0.05). (The remaining patients were treated with chromium-cobalt implants.) Twenty-two (52.4%) of the forty-two patients with deep surgical site infection underwent implant removal, which was complete in thirteen and partial in nine. Growing-rod treatment was terminated in two patients with partial removal and six patients with complete removal. An increased risk of deep surgical site infection was associated with stainless-steel implants (odds ratio [OR] = 5.7), non-ambulatory status (OR = 2.9), and the number of revisions before the development of deep surgical site infection (OR = 3.3). Neuromuscular etiology and non-ambulatory status increased the possibility of implant removal to treat infection (p < 0.05)., Conclusions: The prevalence of deep surgical site infection associated with growing-rod surgery is higher than that associated with standard pediatric spinal fusion (historical data). Non-ambulatory status, more revisions, and stainless-steel implants increased the risk of deep surgical site infection. After eight surgical procedures, the risk of deep surgical site infection increased to approximately 50%. When patients have implant removal, efforts should be made to retain one longitudinal implant to continue treatment., Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2014 by The Journal of Bone and Joint Surgery, Incorporated.)

The management of spinal deformity in children with univentricular cardiac pathology poses significant challenges to the surgical and anaesthetic teams. To date, only posterior instrumented fusion techniques have been used in these children and these are associated with a high rate of complications. We reviewed our experience of both growing rod instrumentation and posterior instrumented fusion in children with a univentricular circulation. Six children underwent spinal corrective surgery, two with cavopulmonary shunts and four following completion of a Fontan procedure. Three underwent growing rod instrumentation, two had a posterior fusion and one had spinal growth arrest. There were no complications following surgery, and the children undergoing growing rod instrumentation were successfully lengthened. We noted a trend for greater blood loss and haemodynamic instability in those whose surgery was undertaken following completion of a Fontan procedure. At a median follow-up of 87.6 months (interquartile range (IQR) 62.9 to 96.5) the median correction of deformity was 24.2% (64.5° (IQR 46° to 80°) vs 50.5° (IQR 36° to 63°)). We believe that early surgical intervention with growing rod instrumentation systems allows staged correction of the spinal deformity and reduces the haemodynamic insult to these physiologically compromised children. Due to the haemodynamic changes that occur with the completed Fontan circulation, the initial scoliosis surgery should ideally be undertaken when in the cavopulmonary shunt stage.

Study Design: Retrospective case series from a multicenter database., Objective: To evaluate the risk of neurologic injury during growing rod surgeries and to determine whether intraoperative neuromonitoring is necessary for all growing rod procedures., Summary of Background Data: Although the use of growing rod constructs for early-onset spinal deformity has become a commonly accepted treatment, the incidence of neurologic events during growing rod surgeries remains unknown., Methods: We reviewed data from a multicenter database on 782 growing rod surgeries performed in 252 patients. VEPTR devices and any constructs with rib attachments were excluded. A questionnaire was sent to all surgeons contributing cases requesting detailed information about all neurologic events associated with any growing rod surgery., Results: There were 782 growing rod surgeries performed on 252 patients including 252 primary growing rod implantations, 168 implant exchanges, and 362 lengthenings. Five hundred sixty-nine of 782 (73%) cases were performed with neuromonitoring. Only one clinical injury occurred in the series, resulting in an injury rate of 0.1% (1/782). This deficit occurred during an implant exchange while attempting pedicle screw placement, and resolved within 3 months. There were 2 cases with neuromonitoring changes during primary implant surgeries (0.9%, 2/231), 1 change during implant exchanges (0.9%, 1/116), and 1 neuromonitoring change during lengthenings (0.5%, 1/222). The single monitoring change that occurred during a lengthening was in a child with an intracanal tumor who also had a monitoring change during the primary surgery. There are anecdotal cases (outside this study group series) of neuromonitoring changes during simple lengthenings in children with uneventful primary implantations., Conclusion: Based on our study, the largest reported series of growing rod surgeries, the rate of neuromonitoring changes during primary growing rod implantation (0.9%) and exchange (0.9%) justifies the use of intraoperative neuromonitoring during these surgeries. As there were no neurologic events in 361 lengthenings in patients with no previous neurologic events, the question may be raised as to whether intraoperative neuromonitoring is necessary for simple lengthenings in these patients. However, caution should be maintained when interpreting our results as anecdotal cases of neurologic changes from simple lengthenings do exist outside of this series.

Study Design: Review article., Objective: To discuss natural history of congenital scoliosis and kyphosis., Summary of Background Data: Review of previously published literature on natural history of congenital spine deformities., Methods: Medline and google search for congenital scoliosis, kyphosis, and kyphoscoliosis, congenital spine anomalies, deformities, and pathologies, and congenital vertebral anomalies, deformities, and pathologies was performed., Results: Congenital vertebral anomalies have potential to progress and careful assessment and monitoring is essential and early intervention may be desirable., Conclusion: Congenital vertebral anomalies invariably result from disturbed asymmetric growth and can have serious consequences.

Anterior scoliosis surgery is associated with potentially high blood loss, usually requiring allogenic transfusion either intra- or post-operatively. Blood loss in this type of surgery has been shown to correlate with surgical and anaesthetic techniques. In our centre the development of specific anaesthetic techniques as well as the routine use of cell salvage has dramatically reduced the rates of allogenic blood transfusion. Specific indications for the use of the cell saver in anterior scoliosis surgery have not been well defined. Previous studies have commented on the benefit from re-infusion of salvaged autologous blood for orthopaedic patients in general, whilst others have shown a negligible advantage specifically in anterior thoraco-lumbar fusion surgery. We carried out a retrospective study of 137 consecutive patients, all of whom underwent instrumented anterior scoliosis correction between March 1999 and September 2004. A study group consisting of 104 patients in whom a cell saver was used was compared with a control group consisting of 33 patients who underwent anterior instrumentation without cell saver. There was no significant difference in the mean ages, extent of surgery and male to female ratio between groups. In the control group 39.4% of patients required allogenic blood transfusion, versus 6.7% in the study group; the difference is statistically significant (p < 0.0001). A significant difference was also noted in post-operative haemoglobin values. The mean post-operative haemoglobin was 9.6 g/dl in the control group, versus 10.2 g/dl in the study group (p = 0.007). Our experience confirms that re-infusion of salvaged autologous blood in anterior scoliosis surgery has a role in the minimisation of postoperative anaemia and allogenic transfusion requirements in this type of surgery.

Study Design: Retrospective case review of children completing dual growing rod treatment at our institutions. Patients had a minimum of 2 years follow-up., Objective: To identify the factors influencing dual growing rod treatment outcome followed to final fusion., Summary of Background Data: Published reports on dual growing rod technique results for early onset scoliosis demonstrate it to be safe and effective in curve correction and maintenance as well as in allowing spinal growth., Methods: Between 1990 and 2003, 13 patients with no previous surgery and noncongenital curves underwent final fusion. All had preoperative curve progression over 10 degrees after unsuccessful nonoperative treatment. There were 10 females and 3 males. Average age was 6.6 +/- 2.9 years at initial surgery. There were 3 idiopathic, 1 nonspine congenital anomaly, and 9 syndromic patients. Analysis included age at initial surgery and final fusion, number and frequency of lengthenings, and complications. Radiographic evaluation included changes in Cobb angle, T1-S1 length, and instrumentation length over the treatment period., Results: Cobb angle improved from 81.0 +/- 23 degrees to 35.8 +/- 15 degrees postinitial and 27.7 +/- 17 degrees after final fusion. Average number of lengthenings was 5.2 +/- 3 at an interval of 9.4 +/- 5 months. T1-S1 length increased from 24.4 +/- 3.4 to 29.3 +/- 3.6 cm postinitial and 35.0 +/- 3.7 cm postfinal fusion. Average growth was 1.46 +/- 0.66 cm/year. Those lengthened at

Beals syndrome (congenital contractural arachnodactyl) is a genetic disorder of the connective tissue phenotypically related to Marfan syndrome. It is characterised by dolichostenomelia, arachnodactyly, multiple joint contractures, crumpled ears, hypoplastic muscles and scoliosis. The latter, the most important clinical feature of this rare condition, presents in the infantile and juvenile age group and has a tendency to rapid progression. Bracing often fails to control the scoliosis and surgery is the recommended treatment. We present our experience of two cases managed with the paediatric Isola instrumentation and a non-fusion technique.

Study Design: Retrospective analysis of patients treated with single and dual growing rods who had completed their course of treatment, had definitive fusion, and had a minimum of 2 years follow-up., Objectives: To determine which technique was the most effective in the management of severe spinal deformity in young children: control of the spinal deformity, spinal growth, and the incidence of complications., Summary of Background Data: Growing rod techniques provide proximal and distal segmental "claw" foundations, but their overall results through definitive fusion have not been clearly determined., Methods: A total of 28 consecutive patients who had growing rod procedures followed through definitive spinal fusions were analyzed. There were three patient groups: Group 1 (N = 5), single submuscular rod and short apical fusion; Group 2 (N = 16), single growing rod alone; and Group 3 (N = 7), dual growing rods., Results: The interval between initial rod insertion and definitive spinal fusion was similar in all three groups. The best overall results occurred in Group 3, whereas the patients in Group 1 had the worse results. Both Groups 2 and 3 provided good initial correction of the spinal deformity and allowed spinal growth. Group 2 had better frontal and sagittal plane balance and the lowest complication rate., Conclusion: The use of growing rods is effective in controlling severe spinal deformities and allowing spinal growth. Dual rods are stronger than single rods and, therefore, provide better initial correction and maintenance of correction. The use of an apical fusion does not appear to be effective over the course of treatment.

Study Design: A retrospective case review of children treated with dual growing rod technique at our institutions. Patients included had no previous surgery and a minimum of 2 years follow-up from initial surgery., Objectives: To determine the safety and effectiveness of the previously described dual growing rod technique in achieving and maintaining scoliosis correction while allowing spinal growth., Summary of Background Data: Historically, the growing rod techniques have used a single rod and the reported results have been variable. There has been no published study exclusively on the results of dual growing rod technique for early-onset scoliosis., Methods: From 1993 to 2001, 23 patients underwent dual growing rod procedures using pediatric Isola instrumentation and tandem connectors. Diagnoses included infantile and juvenile idiopathic scoliosis, congenital, neuromuscular, and other etiologies. All had curve progression over 10 degrees following unsuccessful bracing or casting. Of 189 total procedures within the treatment period, 151 were lengthenings with an average of 6.6 lengthenings per patient. Analysis included age at initial surgery and final fusion (if applicable), number and frequency of lengthenings, and complications. Radiographic evaluation included measured changes in scoliosis Cobb angle, kyphosis, lordosis, frontal and sagittal balance, length of T1-S1 and instrumentation over the treatment period, and space available for lung ratio., Results: The mean scoliosis improved from 82 degrees (range, 50 degrees-130 degrees) to 38 degrees (range, 13 degrees-66 degrees) after initial surgery and was 36 degrees (range, 4 degrees-53 degrees) at the last follow-up or post-final fusion. T1-S1 length increased from 23.01 (range, 13.80-31.20) to 28.00 cm (range, 19.50-35.50) after initial surgery and to 32.65 cm (range, 25.60-41.00) at last follow-up or post-final fusion with an average T1-S1 length increase of 1.21 cm per year (range, 0.13-2.59). Seven patients reached final fusion. The space available for lung ratio in patients with thoracic curves improved from 0.87 (range, 0.7-1.1) to 1.0 (range, 0.79-1.23, P = 0.01). During the treatment period, complications occurred in 11 of the 23 patients (48%), and they had a total of 13 complications. Four of these patients (17%) had unplanned procedures. Following final fusion, 2 patients required extensions of their fusions because of curve progression and lumbosacral pain., Conclusion: The dual growing rod technique is safe and effective. It maintains correction obtained at initial surgery while allowing spinal growth to continue. It provides adequate stability, increases the duration of treatment period, and has an acceptable rate of complication compared with previous reports using the single rod technique.

Study Design: A prospective clinical study in which autologous rib graft, harvested during the thoracotomy in staged scoliosis correction, is stored within the patient for use during the second stage (posterior intrumentation and fusion)., Objective: To determine whether the bone stored by this technique is biologically viable and microbiologically safe., Summary of Background Data: To the authors' knowledge, this method of storage of bone has never been described previously., Methods: During the first operation, the excised rib was divided into 3-5 cm fragments and stored in a sub-muscular plane adjacent to the posterior elements of the spine before closure. The graft was then retrieved at the second stage. Samples were sent for histologic and microbiologic examination before implantation., Results: On histologic examination, more than 50% of the osteocytes retained their basophilic staining, indicating that they were viable. In addition, osteoclastic activity was notably absent. There was no significant bacterial contamination of the samples. Clinically, all patients achieved satisfactory bone fusion., Conclusion: Homeostatic equilibrium in humans provides the ideal environment in which bone graft can be stored. There is no increased risk of infection, and the osteogenic potential of the graft is retained.

Study Design: Retrospective review of patient records with current clinical and radiographic assessment., Objective: To evaluate the long-term result of anterior and posterior convex spinal growth arrest, with or without instrumentation, in managing infantile idiopathic scoliosis., Summary of Background Data: There were 12 male and 10 female patients studied, with a mean follow-up period of 10 years, 9 months. The mean Cobb angle before surgery was 65 degrees. All had a rib vertebral angle difference more than 20 degrees. The mean age at surgery was 6 years. Nine patients had epiphysiodesis alone; nine patients also underwent Harrington instrumentation simultaneously, and four underwent Harrington instrumentation 2-4 years later., Method: Clinical evaluation and sequential measurements of Cobb angle were done., Results: The epiphysiodesis-only group had a mean preoperative Cobb angle of 72 degrees, mean progression of curves of +12 degrees, and mean rate of progression of +2.5 degrees per year: the group's postoperative figures were 92 degrees, +15 degrees, and +3 degrees per year, respectively. The epiphysiodesis and late Harrington rod group had a mean preoperative Cobb angle of 56 degrees, mean progression of +12 degrees, and a mean rate of progression of +5 degrees per year; the group's postoperative Cobb angle averaged 62 degrees, progression +6 degrees, and rate of progression +1 degree per year. The epiphysiodesis with simultaneous Harrington rod group had a preoperative mean Cobb angle of 60 degrees, mean progression of +18 degrees, and mean rate of progression of +6 degrees per years. After surgery, these improved to 58 degrees, correction of 2 degrees, and rate of correction of 0.5 degree per year., Conclusion: Combined anterior and posterior convex spinal growth arrest alone does not prevent progression of deformity in infantile idiopathic scoliosis. The addition of posterior instrumentation can slow or arrest deformity progression but not reverse it.

Study Design: Retrospective review of patient records with recent clinical and radiologic assessment., Objective: To evaluate the long-term result of anterior and posterior convex ephiphysiodesis in the management of congenital scoliosis resulting from fully segmented nonincarcerated hemivertebra., Summary of Background Data: Thirty patients (16 male/14 female patients) were reviewed. Follow-up was a minimum of 3 years (average, 8 years 10 months; range, 3-22.5 years). Nineteen patients were skeletally mature, and the mean age of the remaining 11 was 11.75 years., Method: Clinical evaluation and sequential measurements of Cobb angle was made independently by two observers., Results: Compared with preoperative values, the rate of change in Cobb angle was reversed in 23 patients, arrested or slowed in five patients, and unchanged or progressed in two patients. The annual rate of change in Cobb angle was +1.9 degrees before surgery and -1.2 degrees after surgery, a difference of 3.1 degrees. This is highly statistically significant (P < 0.001). Total correction in Cobb angle correlates with age at time of surgery (P < 0.03). The rate of correction in Cobb angle after surgery correlates with the total correction achieved (P < 0.001) and with age at time of surgery (P < 0.05). The greater correction is achieved when surgery is performed at a young age. The preoperative rate of increase in Cobb angle does not correlate with correction (P < 0.76). The site of the hemivertebrae influenced final outcome with best results in the lumbar spine., Conclusion: Combined anterior and posterior convex epiphysiodesis is a reliable method for the correction of deformity resulting from hemivertebrae.

The objective of this study was to evaluate the long term outcome of combined anterior and posterior convex spinal epiphysiodesis in the treatment of congenital scoliosis. The study covered 53 patients (27 male, 26 female) with a minimum follow up period from surgery of 3 years (mean 8.8 years, range 3-22.5 years). Of these, 34 were skeletally mature when reviewed. Clinical assessment and sequential measurement of Cobb angles were used to chart the course of the deformity following surgery. The types of vertebral anomalies encountered were: 4 unsegmented bars, 7 unsegmented bars with hemivertebrae, 30 hemivertebrae (of which 2 were double hemivertebrae) and 12 complex (unclassifiable) patterns. The severe types were concentrated in the thoracic spine. Results are presented with reference to the type and site of anomaly and to the age of the patient at the time of surgery. Where deformity was due to an unsegmented bar (with or without hemivertebra) the rate of change of Cobb angle was slowed, but not reversed, following surgery. For the complex anomalies there was a reduction in the rate of progression of deformity following surgery, however, the final Cobb angle still increased from a mean of 61 degrees to 70 degrees. In contrast, the rate of progression reversed or slowed in 97% of the hemivertebra patients following surgery, producing a change in mean Cobb angle from 41 degrees pre-operatively to 35 degrees post-operatively. For each type of anomaly the correction achieved was greater where the surgery was performed at a younger age. Final outcome was influenced by the site of anomaly, with a better correction being achieved in the lumbar than the thoracic spine. In conclusion, we feel that convex epiphysiodesis has an important role in the surgical management of congenital scoliosis and, for hemivertebrae in particular, it can produce significant correction of deformity.