Impact of Spinal Surgery on Intrathecal Nusinersen Injections in Pediatric Spinal Muscular Atrophy.

BACKGROUND: Complex spinal deformities necessitate surgical interventions that may intervene with intrathecal injections in patients with spinal muscular atrophy (SMA). This study aimed to determine the effect of spinal deformity correction surgery on nusinersen administration. METHODS: Pediatric patients with SMA, operated by a single surgeon, either via magnetically controlled growing rod (MCGR) or definitive fusion (DF) with skip instrumentation, were evaluated retrospectively in terms of safety and feasibility of intrathecal injections. Patients' and their parents' perspectives were evaluated through a questionnaire regarding any shift in the setting of injections. RESULTS: Fourteen patients with 15 spinal surgeries (10 MCGR and 5 DF) were included. Eleven patients received intrathecal treatment both before and after the surgery. Preoperative (n=3) and postoperative (n=9) fluoroscopic guidance was required leading to a shift in the application settings in 6 patients. Of 106 preoperative injections, 15% required fluoroscopy and 2% required anesthesia. Postoperatively, of 88 injections, 73% required fluoroscopy and 26% required anesthesia. No patients discontinued intrathecal injections due to technical difficulties associated with the spinal surgery. CONCLUSIONS: This study demonstrates that spinal surgery does not prevent safe and successful intrathecal nusinersen injections. In the DF group, the skip instrumentation technique provided access to interlaminal space for intrathecal injections. In either surgical group, no further auxillary approach was required. Modifications in the injection technique require an institutional approach, and concerns of patients and their families should be addressed accordingly. LEVEL OF EVIDENCE: IV.

Parent-patient Discrepancies in the Quality of Life Assessment of Early-onset Scoliosis: A Comparison Between 2 Questionnaires Completed on 2 Different Time Points - A Preliminary Report.

BACKGROUND: EOSQ-24 is a parent proxy questionnaire designed to assess the health-related quality of life (HRQoL) of early-onset scoliosis (EOS) patients during their childhood years. EOSQ-SELF, a novel self-reported questionnaire, assesses HRQoL in older children (>8 y) and adolescents. So far, the same group of EOS patients has not been evaluated with both EOSQ-24 and EOSQ-SELF. The aim of this study was to evaluate how the same pathology was reflected in the parent and patient at different time points by comparing the answers to the common questions between EOSQ-24 and EOSQ-SELF. METHODS: A group of otherwise healthy EOS patients whose parents filled out EOSQ-24 at the early phase of growth-friendly treatment was re-tested by the EOSQ-SELF questionnaire at the end of treatment. Both EOSQ-24 and EOSQ-SELF are validated in Turkish. Inclusion criteria were patients with EOS, independent ambulation, age of 8 years or older at EOSQ-SELF enrollment, literacy in Turkish, no apparent intellectual impairment, and a minimum of 24 months after graduation. The common questions between the 2 surveys with nearly identical phrasings were extracted. Common items from the 2 tests were compared with a Wilcoxon signed rank test. RESULTS: Twenty-one patients (15 females, 6 males) who previously filled out EOSQ-24 met the inclusion criteria. The mean age of the group was 10 (5 to 16) years at EOSQ-24 participation and 18 (13 to 24) at the final analysis. Fourteen questions were found common in 10 domains. The scores were significantly different in 5 questions of 4 domains. EOSQ-SELF had significantly less favorable scores in the pain/discomfort, pulmonary function, and fatigue/energy level domains. Scores in the parental burden/relationships domain were significantly higher (P<0.05). CONCLUSIONS: The self-reported group had a general trend of worse results. Parents and caregivers may not accurately perceive the problems of EOS patients. Our findings indicate a disconnect between caregivers and the patients, as both parties underreported the other side in some domains. These findings suggest the challenges faced by EOS patients are not adequately reflected on proxy questionnaires that assess the HRQoL of children. LEVEL OF EVIDENCE: Diagnostic Level I.

An Eastern Europe and Middle East multinational expert Delphi consensus study on the prevention, diagnosis, and treatment of developmental dysplasia of the hip before walking age.

PURPOSE: The incidence of developmental dysplasia of the hip (DDH) is higher in Eastern Europeans and Middle Easterners. This study aimed to establish consensus among experts in this geographical area on the management of DDH before walking age. METHODS: Fourteen experienced orthopedic surgeons agreed to participate in a four-round online consensus panel by the Delphi method. The questionnaire included 31 statements concerning the prevention, diagnosis, and treatment of DDH before walking age. RESULTS: Consensus was established for 26 (84%) of 31 statements. Hip ultrasonography is the proper diagnostic tool under six months in DDH; universal newborn hip screening between three and six weeks is necessary; positive family history, breech presentation, female gender, and postnatal swaddling are the most important risk factors; Ortolani, Barlow tests, and limitation of abduction are the most important clinical findings; Pavlik harness is the first bracing preference; some Graf type IIa hips and all Graf type IIb and worse hips need abduction bracing treatment; the uppermost age limit for closed and open reductions is 12 months and 12-24 months, respectively; anatomic reduction is essential in closed and open reductions, postoperative MRI or CT is not always indicated; anterior approach open reduction is better than medial approach open reduction; forceful reduction and extreme positioning of the hips (> 60° hip abduction) are the two significant risk factors for osteonecrosis of the femoral head. CONCLUSION: The findings of the present study may be useful for clinicians because a practical reference, based on the opinions of the multinational expert panel, but may not be applicable to all settings is provided.

Does the Sleeper Plate Application for Temporary Epiphysiodesis Make Life Easier or Complicated? Increased Risk of Tethering.

BACKGROUND: The present study aims to investigate the frequency of recurrence and tethering effect after only metaphyseal screw removal (sleeper plate technique) compared with the conventional complete plate removal in the treatment of lower extremity deformities with guided growth surgery. METHODS: Seventy-two patients (107 limbs) treated by an 8-plate hemiepiphysiodesis technique around the knee joint were evaluated. After the desired correction, only metaphyseal screw was removed (sleeper plate group) in 35 limbs (25 patients), whereas both screws and plate were removed (plate removal group) in 72 limbs (47 patients). An increase of 5 degrees or more in joint orientation angles in the direction of the initial deformity was considered as recurrence. The rate of rebound, tethering, and maintenance of correction in groups was analyzed at the latest follow-up (mean of 49 mo). RESULTS: The mean age of the patients was 97 months (range: 80 to 129 mo) at the time of index surgery. After a mean of 49 months (range: 16 to 86), 17 (48.5%) limbs maintained the desired stable correction in the sleeper plate group compared with 59 stable limbs (72.2%) in the plate removal group ( P <0.001). There was no statistically significant difference regarding recurrence between the sleeper plate group and the plate removal group (34.3% vs. 27.8%, respectively) ( P =0.216). Reinsertion of the metaphyseal screw was possible 8/12 limbs, and the remaining 4 limbs underwent further surgeries. There were 6 limbs (17.3%) of tethering in the sleeper plate group, and 4/6 limbs required further corrective surgeries. The remaining 2 limbs with slight tethering did not require further surgeries. CONCLUSIONS: Removing only metaphyseal screw increases the risk of tethering. In addition, reinsertion of the screw may not be possible in all cases due to bony growth, and further corrective surgeries may be necessary. Close follow-up is required if the sleeper plate technique is to be applied. LEVEL OF EVIDENCE: Level III.

A Comprehensive Assessment of Psychosocial Well-being Among Growing Rod Graduates: A Preliminary Investigation.

BACKGROUND: Traditional growing rods (TGR) encompass a long process, in which patients experience physical and psychosocial difficulties. However, the effect of repeating surgeries on the overall psychological functioning of graduated patients has not been thoroughly investigated in the literature. The aim of this study is to evaluate the psychological well-being of graduated idiopathic early-onset scoliosis patients in terms of psychopathology, neurocognition, and psychosocial functioning, and determine the accuracy of scoliosis outcome questionnaires in these regards. METHODS: TGR graduates with idiopathic early-onset scoliosis without known intellectual disabilities or neuromuscular impairments were included. Patients were thoroughly evaluated using psychological instruments [Wechsler Adult Intelligence Scale, Auditory Consonant Trigram Test, Verbal Fluency Test, Beck Depression Inventory, Beck Anxiety Inventory, Rosenberg Self-Esteem Scale, Symptom Checklist-90, Post-Traumatic Growth Inventory, Strengths and Difficulties Questionnaire, Spinal Appearance Questionnaire, Scoliosis Research Society 22-item questionnaire (SRS-22)]. Results were compared with normative data when available. Spearman correlations were performed between the results of these tests, the total treatment duration, and the number of spinal surgeries. RESULTS: Of the 15 patients included in the study, 9 were females, and the mean age was 18.73 (16 to 23). The mean age at index surgery was 6.38 (3 to 10) whereas that of graduation was 14.00 (12 to 16). The average number of spinal surgeries was 14.28 (7 to 20). Two patients performed below the range of adult intellectual functioning. Auditory Consonant Trigram Test showed normal verbal working memory and attention control. Six patients had abnormal Verbal Fluency Test performance. Eight patients had abnormal ratings on at least one of the assessment scales of psychopathology (Symptom Checklist-90, Beck Depression Inventory, and Beck Anxiety Inventory). Eight patients had low-to-moderate self-esteem (Rosenberg self-esteem scale). The median spinal appearance questionnaire and SRS-22 scores were 34 and 4.18, respectively. Pain and function subdomains of SRS-22 scored higher than self-image and mental health. No correlation was found between the treatment duration and number of surgeries and test scores. SRS-22 showed correlations with multiple psychological tests. CONCLUSION: Completed TGR treatment yields acceptable correction of deformities and surgical outcomes, however, may fail to improve psychological well-being. This is the first study to find various psychosocial abnormalities in two-thirds of cases. LEVEL OF EVIDENCE: Level IV; cross-sectional study.

The Effect of Apical Vertebra Position on Growing Rod Treatment: A Clinical and Finite Element Study.

BACKGROUND: Growing rods (GRs) is a commonly utilized technique for the management of early-onset scoliosis. The significance of the position of the apical vertebrae relative to the rods is not known. The purpose of this study is to analyze the potential effects of the position of the apical vertebrae in relation to the GRs on deformity control through plain radiographs and finite element analysis (FEA) modeling. METHODS: We identified 140 patients treated with GR between 2000 and 2018. Patients who had a congenital vertebral anomaly or <2-year follow-up were excluded. Curve magnitude, traction radiograph under general anesthesia (TRUGA) flexibility, apical rotation, the lengths of T1-12, T1-S1, and the instrumented segments were recorded. Patients were divided into 3 groups according to the apical position on the postoperative radiographs: group 1 (both pedicles are between the rods), group 2 (convex rod is between the apical vertebra pedicles), group 3 (both pedicles are lateral to the convex rod). FEA models were created simulating the 3 groups. Both radiographic and FEA data were analyzed to compare the deformity control and growth in each group. RESULTS: Fifty-eight patients were included in the final analyses (mean age 84 mo; range: 38 to 148). Ten patients (17%) were in group 1, 34 (59%) in group 2, and 14 (24%) in group 3. Difference between TRUGA flexibilities was statistically insignificant. Group 3 was the least successful in terms of both height gain and rotational control. FEA showed a decrease in rotation and displacement for every group, however, the residual rotation and displacement was highest in group 3. CONCLUSIONS: Bringing the apex in line with the GR increases the capacity of growth preservation as it results in largest height gain and better deformity control. FEA model demonstrated that distraction alone is inadequate for controlling rotation, and with increasing apical translation, residual rotation after distraction also increases. LEVEL OF EVIDENCE: Level III.

Posterior Instrumentation and Fusion in Multiple-segment Adolescent Congenital Scoliosis: When Are Posterior Column and Concave Rib Osteotomies Needed?

BACKGROUND: No clear guidelines exist for appropriate surgical treatment of congenital scoliosis. The spectrum varies from isolated posterior instrumentation and fusion (PIF-only) to vertebral column resections. Multiple posterior column osteotomies (PCOs) with and without concave rib osteotomies have previously been suggested as an alternative to invasive 3-column osteotomies. The aim of the study is to analyze outcomes of spinal surgery in congenital scoliosis patients for a new treatment algorithm based on a traction radiograph under general anesthesia (TruGA). METHODS: Surgical intervention was determined according to flexibility with TruGA: patients with >40% correction underwent PIF-only; patients with <40% of correction underwent PCO. Patients, who had <30% of correction in TruGA and/or more than 5 vertebral levels with failure of segmentation, underwent additional concave rib osteotomies. The radiologic and clinical results were compared. RESULTS: Forty-three patients met inclusion criteria (14M). The mean age was 13.8 (10 to 17) years, average follow-up 67 (24 to 107) months. Fourteen patients were in the PIF-only group, 29 in the PCO group. PCO patients were significantly older (14.5 vs. 12.3, P=0.001). Mean operative time and blood loss of PCO group were significantly greater than those of PIF-only group. Nine patients required concave rib osteotomies. While the curve magnitude of patients in the PIF-only and PCO groups did not differ significantly (54.6 vs. 63 degrees, P=0.067), curve rigidity was significantly higher in the patients of PCO group (51.2% vs. 32%, P=0.001). Despite this, postoperative correction rates for 2 groups were similar (51.1% vs. 47.8%, P=0.545). CONCLUSIONS: The number of anomalous segments and the curve flexibility on TruGA play important roles in the decision-making process in congenital scoliosis and <40% flexibility of the major curve could be an important indicator of the need for PCO. Curves with more than 5 anomalous vertebral segments might be more likely to need additional concave rib osteotomies for adequate correction. LEVEL OF EVIDENCE: Level III.

Clinical Outcomes of 3-column Osteotomy at Growing Rod Graduation.

BACKGROUND: Several different strategies were described on how to graduate a patient when skeletal maturity is reached after growing rod treatment. A final instrumentation and fusion is commonly performed, but the stiffness of the spine after numerous lenghthenings can be a major problem. When correction is needed in a severe and rigid curve, 3-column osteotomies (3CO) can be used as a method of bringing the spine back to a well-balanced position. This study aims to evaluate the clinical outcomes of 3CO osteotomies at growing rod graduation. METHODS: A retrospective search of the database was performed between 1996 and 2018. Inclusion criteria were: diagnosis of early-onset scoliosis, underwent 3CO at the final fusion procedure, and ≥1 year of follow-up. Indications for this osteotomy were: significant sagittal and/or coronal off-balance or severe crankshaft deformity or significant distal and/or proximal adding-on. Clinical, radiographic, and operative data were analyzed. Complications were classified according to the modified Clavien-Dindo-Sink system. RESULTS: Eight patients were included in the study with a mean age of 13.5 at graduation (male/female: 2/6). The mean scoliosis angle significantly reduced from 65.6 to 39 degrees (P=0.012). Mean T1-T12 height increased from 19 to 20.5 (P=0.084) and T1-S1 from 29.6 to 31.6 (P=0.144). Five patients had a staged surgery. Average operative time was 338 minutes and blood loss was 1321 mL. Four grade 1, 1 grade 2, and 1 grade 3 complications occurred. CONCLUSIONS: This is the first case-series in the literature reporting on the use of 3CO at growing rod graduation. These procedures can be safely and effectively used to overcome significant multiplanar deformity and/or off-balance in the graduation of patients with early-onset scoliosis and significant correction can be achieved on these severe and rigid curves. LEVEL OF EVIDENCE: Level IV-case series.

Improving Safety and Efficacy in the Surgical Management of Low-tone Neuromuscular Scoliosis: Integrated Approach With a 2-attending Surgeon Operative Team and Modified Anesthesia Protocol.

BACKGROUND: Substantially increased operative time and amount of bleeding may complicate the course of surgical treatment in neuromuscular scoliosis. A well-organized team approach is required to reduce morbidity. The aim of this study is to review our early, short-term surgical outcomes with our new integrated approach that includes a 2-attending surgeon team and modifications in the anesthesia protocol in low-tone neuromuscular scoliosis and compare with a matched cohort of our historic patients. METHODS: We retrospectively reviewed our patients with (1) neuromuscular scoliosis with collapsing spine deformity, (2) low-tone neuromuscular etiology, (3) multilevel posterior column osteotomies with posterior all pedicle screw spinal fusion, and (4) more than 1-year follow-up. Patients were grouped into 2: group 1 consisted of patients managed with the integrated surgical team approach, group 2 included the matched historic patients. RESULTS: There were 16 patients in group 1 and 17 patients in group 2. There was no significant difference between the groups regarding age, sex, body mass index, number of levels fused, major coronal deformity magnitude, pelvic obliquity, number of posterior column osteotomies, or amount of deformity correction. However, significantly shorter operative time (241 vs. 297 min, P=0.006), less intraoperative bleeding (1082 vs. 1852 mL, P=0.001), less intraoperative blood transfusion (2.1 vs. 3.1 U, P=0.028), less postoperative intensive care unit admission (23% vs. 100%, P=0.001), and shorter hospital stay (4.7 vs. 5.9 d, P=0.013) were observed in group 1. CONCLUSIONS: Our results indicate that spinal deformity surgery in patients with underlying low-tone neuromuscular disease may not be as intimidating as previously thought. Our surgical team approach integrating a 2-attending surgeon operative team, a new anesthetic protocol that includes a modification of perioperative blood management is effective in reducing operative times, blood loss, transfusion rates, intensive care unit admission, and length of hospital stay. LEVELS OF EVIDENCE: Level III-retrospective comparative study.

Early Results of a Management Algorithm for Collapsing Spine Deformity in Young Children (Below 10-Year Old) With Spinal Muscular Atrophy Type II.

BACKGROUND: Progressive C-shaped scoliosis with marked pelvic obliquity is common to spinal muscular atrophy (SMA). Reducing the number of procedures with effective deformity control is critical to minimize the risk of pulmonary complications. This study reports the preliminary results of magnetically controlled growing rods (MCGR) in SMA-related collapsing spine deformity. METHODS: Inclusion criteria for this retrospective review were: (1) SMA type 2 patients, (2) early onset scoliosis (below 10 y), (3) collapsing spine deformity with pelvic obliquity, (4) growth-friendly scoliosis treatment with MCGR, (5) in between 2014 and 2017. Extracted data included demographic and clinical information, radiologic parameters, surgical details, and final status of the patients. RESULTS: A total of 11 patients (7 boys, 4 girls) were included. The average age at index surgery was 8.2 (6 to 10) years. Dual MCGR was implanted in 8 patients. In 3 patients, because of curve rigidity and inability of apex to be brought into the stable zone, apical fusion with gliding connectors (convexity) and a single MCGR (concavity) was preferred. Instrumentation included the pelvis in 9 and stopped at the lumbar spine (L3) in 2 patients at the index procedure. Average preoperative deformity of 81.8 degrees (66 to 115) decreased to 29 degrees (11 to 57) postoperatively and was 26 degrees at average 35 months (16 to 59). Pelvic obliquity of 20.9 degrees (11 to 30) decreased to 4.9 degrees (2 to 8) after index surgery and was 6.5 degrees (2 to 16) at the last follow-up. T1-S1 height of 329 mm (280 to 376) after index surgery increased to 356 mm (312 to 390) after 9.2 (4 to 20) outpatient lengthening. No neurologic, infectious, or implant-related complication was recorded. Distal adding-on deformity occurred in 2 patients without initial pelvic fixation.One patient deceased secondary to pneumonia at 16 months after surgery. CONCLUSIONS: Short-term results indicate that MCGR may be a good option in SMA-associated collapsing spine deformity to reduce the burden of repetitive lengthening procedures. The authors recommend apical deformity control in the convex side in case of curve rigidity. In addition, including the pelvis in the instrumentation at index surgery is critical to prevent distal adding-on. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Growing-rod Graduates With Idiopathic Early-onset Scoliosis Have Comparable Exercise Tolerance to Patients With Surgically Treated Adolescent Idiopathic Scoliosis.

BACKGROUND: Growing-rod (GR) treatment is the current standard for progressive idiopathic early-onset scoliosis (I-EOS) in young children. Despite good radiographic outcomes, the impact of scoliosis treatment on pulmonary functions is not well-defined in this patient population. The aim of this study was to evaluate pulmonary functions and exercise tolerance in I-EOS patients graduated from GR treatment and to compare them with age-matched, surgically treated adolescent idiopathic scoliosis (AIS) patients and healthy controls. METHODS: Eight GR graduates with I-EOS with pulmonary function tests and complete radiographic results were compared with a group of 9 thoracic AIS patients at least 2 years out from posterior fusion. Both groups were also compared with a set of 10 healthy individuals. All subjects underwent cardiopulmonary exercise testing and spirometry to evaluate pulmonary function. RESULTS: Age, sex, height, arm span, weight, residual deformity, and level of instrumentation in GR and AIS patients were similar. In the GR group, forced vital capacity % and forced expiratory volume in 1 second % values were reduced compared with the healthy controls and AIS group (P<0.001, <0.001 and 0.036, 0.046, respectively). Breathing reserve index at lactate threshold (BRILT) was higher in GR and AIS patients (P=0.001 and 0.002, respectively), and was similar between GR and AIS patients (P=0.916). Heart rate at lactate threshold was higher in GR and AIS groups compared with controls (P<0.001 and 0.001, respectively). CONCLUSIONS: AIS and GR patients demonstrated reduced pulmonary reserve and exercise tolerance compared with their peers with no spinal deformity. However, exercise tolerance of I-EOS patients treated with the GR method was similar to that of operated AIS patients. These results suggest a positive impact of GR treatment in children with I-EOS. LEVEL OF EVIDENCE: Level III-cross-sectional comparative study.

Is Magnetically Controlled Growing Rod the Game Changer in Early-onset Scoliosis? A Preliminary Report.

BACKGROUND: Magnetically controlled growing rod (MCGR) concept was introduced with the premise of minimizing the repetitive lengthening surgeries, which is default in traditional growing rod (TGR) treatment for early-onset scoliosis (EOS). Despite good radiographic outcomes, previous studies did not compare identical patient groups in terms of etiology and deformity characteristics; therefore, a true comparison of the MCGR and TGR is essential. This study was designed to compare 2 techniques in terms of clinical, radiologic, and health-related quality of life (HRQoL) outcomes. METHODS: Patients with long sweeping congenital curves who underwent convex growth arrest and concave distraction (with TGR or MCGR) were retrospectively reviewed. Instrumented all-posterior convex growth arrest and concave distraction with growing rod technique were performed. Demographic parameters, follow-up time, number of lengthening procedures, radiographic parameters, number of unplanned surgeries, and complications were recorded. The Early-Onset Scoliosis Questionnaire (EOSQ-24) was used to evaluate HRQoL outcomes. RESULTS: A total of 20 patients were included (10 MCGR, 10 TGR). No significant differences were found with regard to average age, follow-up time, radiographic parameters, or complications. Overall surgery per patient including index surgery, and planned and unplanned procedures were significantly lower in the MCGR group (8.8 vs. 1.3) (P=0.01). No patient in either group had graduated from growing rod treatment. HRQoL analysis revealed no significant difference between the 2 groups in any specific domain or in the overall score of the EOSQ-24. CONCLUSIONS: Although equally effective in controlling the deformity and superior in reducing the number of surgeries with comparable complication rates, MCGR does not offer any significant improvement in HRQoL outcomes or the overall complication rate. Despite the obvious advantages, according to this preliminary report, the current technology and technique of MCGR may not be sufficient to be the long-awaited "game changer" in the treatment of EOS.

Spinal Instrumentation in Growing Children Retards the Natural Development of Pelvic Incidence.

BACKGROUND: Pelvic incidence increases gradually throughout growth until skeletal maturity. Growing rod instrumentation has been suggested to have a stabilizing effect on the development of the normal sagittal spinal alignment. The purpose of this study is to determine the effect of fixed sagittal plane caused by dual growing rod instrumentation on the natural progression of sagittal spinopelvic parameters in children with idiopathic or idiopathic-like early onset scoliosis. METHODS: Hospital records of children with growing rod instrumentation from 4 separate institutions were reviewed retrospectively. Inclusion criteria were idiopathic or idiopathic-like early onset scoliosis, treatment with dual growing rods with lower instrumented vertebra L4 or upper and more than 2 years of follow-up. Instrumentation levels, magnitudes of major curve, thoracic kyphosis (T2-T12), lumbar lordosis (L1-S1) and pelvic incidence were recorded from preoperative and postoperative standing whole-spine radiographs. Estimated pelvic incidence was also calculated for each patient as if their spines had not been instrumented using the previous normative data. RESULTS: A total of 37 patients satisfied the inclusion criteria. Average age at initial surgery was 7.4±1.8 years (range, 4 to 12 y). Mean follow-up time was 71±26 months (range, 27 to 120 mo). Mean preoperative Cobb angle of 59±13.5 (range, 30 to 86) degrees was reduced to 35.1±17.5 (range, 11 to 78) degrees at the last follow-up. Mean preoperative T2-T12 kyphosis angle was 46.2±14.9 degrees (range, 22 to 84 degrees). At the latest follow-up, it was 44.8±16.2 degrees (range, 11 to 84 degrees) (P=0.93). Mean L1-S1 lordosis angle was 50.5±10.7 degrees (range, 30 to 72 degrees) preoperatively. At the latest follow-up, mean L1-S1 lordosis angle was 48.8±12.7 degrees (range, 26 to 74 degrees) (P=0.29). Mean preoperative pelvic incidence was 45.7±7.9 degrees (range, 30 to 68 degrees). At the latest follow-up, it was 46.7±8.4 degrees (range, 34 to 72 degrees) (P=0.303). The estimated average pelvic incidence was 49.5 degrees (P=0.012). CONCLUSIONS: Previously reported developmental changes of the sagittal spinal parameters were not observed in children who underwent posterior spinal instrumentation. Our findings suggest that spinal instrumentation impedes the natural development of the sagittal spinal profile. LEVEL OF EVIDENCE: Level IV-this is a retrospective case-series.

Surgical Management of Progressive Thoracolumbar Kyphosis in Mucopolysaccharidosis: Is a Posterior-only Approach Safe and Effective?

BACKGROUND: According to the current literature, the recommended surgical treatment is circumferential spinal fusion, including both anterior and posterior procedures, for progressive thoracolumbar kyphosis in mucopolysaccharidosis (MPS). The purpose of this study was to report our experience with the posterior-only approach and instrumented fusion for MPS kyphosis. METHODS: Six consecutive patients with MPS and thoracolumbar junctional kyphosis managed with the posterior-only approach were included. Demographic data, the type of MPS, medical comorbidities, and accompanying clinical manifestations were recorded. Measurements recorded on radiographs for the study included the presence of any coronal-plane deformity, fusion levels, changes in the local kyphosis angle (LKA), proximal and distal junctional kyphosis angles, and the apical vertebral wedge angle. RESULTS: The average age at the time of surgery was 6.6 (range, 4 to 12) years. The average follow-up duration was 52.6 (range, 44 to 64) months. The mean preoperative LKA of 63.1±15.8 (range, 48 to 92) degrees decreased to a mean of 16.6±8.4 (range, 5 to 30) degrees immediately after surgery. At the latest follow-up, the mean LKA was 19.6±8.8 (range, 8 to 34) degrees. Apical vertebral listhesis was reduced in all patients with surgical correction. The average apical vertebral wedge angle of 15 (range, 11 to 19) degrees at the early postoperative period decreased to 4.6 (range, 2 to 7) degrees at the latest follow-up. Adjacent-segment problems occurred in 2 patients. There were no neurological complications or implant failures in any of the patients. CONCLUSIONS: Posterior-only corrective techniques and instrumented fusion with pedicle screws for progressive thoracolumbar junctional kyphosis in MPS patients are safe and effective methods. Its results are comparable to those achieved with conventional circumferential fusion. However, patients should be monitored closely for adjacent-segment problems. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Pull-out of Upper Thoracic Pedicle Screws Can Cause Spinal Canal Encroachment in Growing Rod Treatment.

BACKGROUND: Proximal foundation failure is a common complication of growing rod (GR) treatment for early-onset scoliosis. Spinal canal encroachment due to pull-out of pedicle screw used as proximal foundation has been anecdotally reported in GR patients. The aim of this study is to report the prevalence of spinal canal encroachment of pedicle screws in GR treatment and determine risk factors using a single-center cohort. METHODS: Inclusion criteria were: (1) GR for early-onset scoliosis and (2) pull-out of at least 1 proximal anchor pedicle screw. Patients were divided into 2 groups according to the presence of medial screw migration. Medial migration of the screw was confirmed by computed tomography. The extracted data included demographic, clinical, and radiographic information. RESULTS: A total of 21 patients (of 96) met inclusion criteria (21.8%). None of the screws appeared malpositioned on early postoperative x-ray. Average follow-up until screw failure was 50.4 months (64 to 85 mo) and average number of lengthenings 8.1 (4 to 13). Computed tomography revealed canal encroachment in 11 patients (group 1), and no encroachment in 10 (group 2). There was no significant difference between groups for age, follow-up or number of lengthenings. At the time of screw pull-out, coronal plane deformity was increased compared with early postoperative x-ray in all; however, this increase was significantly higher in group 1 (45.7 vs. 35 degrees, P=0.002). Proximal junctional angle (PJA) was increased in both groups at the time of pull-out. While not statistically significant, PJA increased linearly in group 1 but spiked in group 2 at the time of pull-out. There was no neurological event preoperatively, intraoperatively or postoperatively. Failed screws were safely revised in either planned/unplanned surgeries. CONCLUSIONS: In patients with proximal anchor failure of GR, especially if there is increase of coronal deformity and/or PJA, possible spinal encroachment should be kept in mind. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Advances in growing rods treatment for early onset scoliosis.

PURPOSE OF REVIEW: The goal of treatment in early onset scoliosis is to correct the deformity while allowing the thoracic growth for optimal cardiopulmonary functions. Growing rods treatment is a distraction-based, growth-friendly method that is commonly used in treatment of early onset scoliosis with its specific indications. Magnetically controlled growing rods (MCGR) method has been introduced to avoid morbidity of recurrent lengthening procedures. In this review, recent developments in traditional growing rods and MCGR are summarized. RECENT FINDINGS: As the experience with growing rods increased and favorable results were reported, its indications have expanded. Recent studies focused on patient outcomes and complications. Another area of interest is the effects of growing rods in the sagittal spinal alignment. Midterm results demonstrated that MCGR treatment is promising but not free of complications. In MCGR, there is no consensus on the frequency and amount of distraction per session. Rod contouring and behavior of the magnet under MRI are major issues. SUMMARY: Growing rods treatment successfully controls the deformity while preserving the growth of spine and thorax, despite high complication rates. Magnetically controlled systems are introduced to avoid repetitive surgical lengthening procedures. Although preliminary results are promising, there are still significant challenges and unknowns for MCGR.

Convex Instrumented Hemiepiphysiodesis With Concave Distraction: A Treatment Option for Long Sweeping Congenital Curves.

BACKGROUND: Convex hemiepiphysiodesis has been reported to have mixed results in the treatment of congenital spine deformities. Multiple modifications of the original technique were suggested to improve the results. The purpose of this study is to report the results and complications of an instrumented convex growth arrest procedure modified with concave distraction. METHODS: The records of 11 patients with long sweeping congenital curves (involving multiple anomalous vertebrae) who underwent convex instrumented hemiepiphysiodesis with concave distraction were evaluated. Mean age at index operation was 58 months (range, 29 to 101 mo). Lengthening of the concave distraction rod was done every 6 months. The magnitude of coronal/sagittal deformity and T1-T12 height were measured on the preoperative, postoperative, and latest follow-up radiographs. Average follow-up was 44.9 months (range, 24 to 89 mo). RESULTS: In the coronal plane, the convex hemiepiphysiodesis segment was corrected from an average of 60.5 to 40.4 degrees postoperatively and further improved to 35.5 degrees at the latest follow-up. The distracted segment was corrected from 33.4 to 15.2 degrees postoperatively and to 12.7 degrees at the latest follow-up. Sagittal plane alignment was minimally affected. The average T1-T12 height was 157.1 mm in the early postoperative period and 181.1 mm at last follow-up. During follow-up, we identified partial pull-out of screws on the distraction side in 5 of the 11 patients and rod breakages in 3 patients. These were revised during planned lengthenings. There were no unplanned surgeries, deep wound infections, nor neurological complications. CONCLUSIONS: Convex instrumented hemiepiphysiodesis with concave distraction resulted in good curve correction while maintaining the growth of thorax. The correction of the anomalous segment improved over time, proving the effectiveness of the hemiepiphysiodesis. Addition of a concave distraction construct appears to enhance spinal growth, thereby augmenting the hemiepiphysiodesis effect.

Utilizing the "Stable-to-be Vertebra" Saves Motion Segments in Growing Rods Treatment for Early-Onset Scoliosis.

BACKGROUND: There is no consensus on the selection of distal instrumentation levels in growing rod surgery. Many surgeons utilize the stable zone of Harrington, but there is not overwhelming evidence to support this preference. The aim of this study was to determine the value of bending/traction radiographs in selection of distal instrumentation levels of a growing rod construct in children with idiopathic or idiopathic-like early-onset scoliosis (EOS). METHODS: Twenty-three consecutive patients with idiopathic or idiopathic-like EOS who underwent growing rod surgery at 2 separate institutions between 2006 and 2011 were included. Lengthening procedures were performed periodically at 6-month intervals. Analyses were performed retrospectively for age at index surgery, follow-up period, and radiographic measurements. Lower instrumented levels, neutral vertebra, stable vertebrae (SV), and stable-to-be vertebrae (StbV) were identified on the preoperative radiographs. Coronal Cobb angles, tilt of lower instrumented vertebra (LIV) and LIV+1, and disk wedging under the LIV and LIV+1 were measured on the early postoperative and latest follow-up radiographs. RESULTS: Average age at index surgery was 83.6 months. Mean follow-up period was 68.1 months. Initial analysis showed that the relationship of LIV to the StbV was a better predictive of LIV+1 tilt than the SV at the final follow-up. Therefore, the patients were grouped according to the relationship of the LIV to the StbV. LIV was the StbV in 9 patients, proximal to the StbV in 8 patients, and distal to the StbV in 6 patients. At the latest follow-up, tilt of LIV+1 exceeded 10 degrees in 7 of the 8 patients where LIV was proximal to the StbV, whereas only in 1 of 9 patients where LIV was StbV, and in none of the 6 patients where LIV was distal to the StbV. The data indicate that selection of the StbV as the LIV could spare an average of 1.8 vertebral segments when compared with the SV, as StbV is never distal but almost always proximal to the SV. CONCLUSIONS: Choosing the StbV as the LIV saves motion segments and prevents distal adding on, while providing satisfactory deformity correction in idiopathic and idiopathic-like EOS. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Convex Hemiepiphysiodesis: Posterior/anterior in-situ Versus Posterior-only With Pedicle Screw Instrumentation: An Experimental Simulation in Immature Pigs.

STUDY DESIGN: Experimental study. BACKGROUND: Convex growth arrest (CGA) has been commonly used in the treatment of long-sweeping congenital deformities of the immature spine. As there are major drawbacks about the anterior procedure in the conventional CGA method, a new modification has been documented that using only posterior spinal approach with pedicle screw instrumentation. The aim of the study was to compare posterior-only CGA using pedicle screws with combined anterior/posterior in-situ CGA for the findings in histologic, radiologic, and manual palpation examinations in an immature pig model. METHODS: Twelve 10-weeks old pigs were grouped into 2. In group 1, posterior-only, pedicle screw instrumented CGA was performed on the left side of L1-L4 vertebrae. In group 2, conventional combined posterior and anterior CGA was performed to the left side of L1-L4 vertebrae without instrumentation. All animals were killed twelve weeks after surgery. T11-L5 segments were en-bloc resected and radiologic, histologic, and manual palpation examinations were done. RESULTS: Marked scoliotic (12.2±2.5 and 9.2±1.3 in group 1 and 2, respectively) and kyphotic (11.2±1.0 degrees for the group 1 and 12±5.2 degrees for the group 2, respectively) deformities were noted in both groups, which were caused by hemiepiphysiodesis effect. Anterior and posterior parts of group 2 and posterior part of group 1 demonstrated fusion in histologic and radiologic analyzes. In anterior part of the group 1, marked narrowing on the disk spaces and thinning of growth plates were noted in radiologicg examination, chondrocyte degeneration, and newly-formed bone trabeculae in disk-space were noted in histological examination. In manual palpation, no motion was detected in group 1 and motion was detected in only one segment of one animal in group 2. CONCLUSIONS: Anterior growth of the vertebrae can be controlled by application of posterior transpedicular screws and rod. Such an effect can eliminate the need for anterior surgical intervention in convex hemiepiphysiodesis procedures. CLINICAL RELEVANCE: The instrumented CGA technique provides a satisfactory epiphysiodesis effect both anteriorly and posteriorly, as previously demonstrated by clinical studies.

Self-growing Instrumentation With Gliding Connectors for Collapsing Spine Deformities in Children: A Novel Technique.

BACKGROUND: The optimal management of young children with neuromuscular spinal deformities is currently unknown. A number of spinal instrumentation techniques have been proposed in early-onset scoliosis to achieve "guided growth," each with its drawbacks. OBJECTIVE: To report a novel self-growing spinal instrumentation technique, designed to avoid recurrent surgeries in children with neuromuscular disease. METHODS: The technique is based on the control of apical and end vertebrae by pedicle screw fixation and limited fusion. Standard 4.5 to 5.5 mm side-to-side rod connectors are used as gliding connections. Three children with neuromuscular disease underwent the described procedure. The patients were followed for an average of 36 months (range, 24 to 60 mo). RESULTS: All patients who underwent the procedure showed an average spinal growth of 1.1 mm/year, which is comparable with normative growth data and previous reports using growing rods. One patient required the exchange of dislodged rods, secondary to the amount of growth of the spine. There were no other complications or unplanned surgeries during the follow-up period. CONCLUSIONS: This novel method provided growth in a group of 3 patients with collapsing neuromuscular spine deformities. LEVEL OF EVIDENCE: Level IV-case series.