Nusinersen therapy changed the natural course of spinal muscular atrophy type 1: What about spine and hip?

Purpose: Spinal muscular atrophy type 1 has a devastating natural course and presents a severe course marked by scoliosis and hip subluxation in nonambulatory patients. Nusinersen, Food and Drug Administration-approved spinal muscular atrophy therapy, extends survival and enhances motor function. However, its influence on spinal and hip deformities remains unclear. Methods: In a retrospective study, 29 spinal muscular atrophy type 1 patients born between 2017 and 2021, confirmed by genetic testing, treated with intrathecal nusinersen, and had registered to the national electronic health database were included. Demographics, age at the first nusinersen dose, total administrations, and Children's of Philadelphia Infant Test of Neuromuscular Disorders scores were collected. Radiological assessments included parasol rib deformity, scoliosis, pelvic obliquity, and hip subluxation. Results: Mean age was 3.7 ± 1.1 (range, 2-6), and average number of intrathecal nusinersen administration was 8.9 ± 2.9 (range, 4-19). There was a significant correlation between Children's of Philadelphia Infant Test of Neuromuscular Disorders score and the number of nusinersen administration (r = 0.539, p = 0.05). The correlation between Children's of Philadelphia Infant Test of Neuromuscular Disorders score and patient age (r = 0.361) or the time of first nusinersen dose (r = 0.39) was not significant (p = 0.076 and p = 0.054, respectively). While 93.1% had scoliosis, 69% had pelvic obliquity, and 60.7% had hip subluxation, these conditions showed no significant association with patient age, total nusinersen administrations, age at the first dose, or Children's of Philadelphia Infant Test of Neuromuscular Disorders scores. Conclusion: Disease-modifying therapy provides significant improvements in overall survival and motor function in spinal muscular atrophy type 1. However, progressive spine deformity and hip subluxation still remain significant problems in the majority of cases which would potentially need to be addressed.

Elastic Stable Intramedullary Nailing in Length Stable Versus Unstable Pediatric Femoral Shaft Fractures: A Comparison of Clinical, Radiographic, and Pedobarographic Outcomes.

BACKGROUND: Pediatric diaphyseal femoral fracture (PDFF) is one of the most common injuries requiring hospitalization. Elastic stable intramedullary nailing (ESIN) is commonly used for PDFFs in ages 5 to 11. The optimal treatment method for length unstable PDFF is a subject of ongoing debate. This study aimed to compare clinical, radiographic, and pedobarographic outcomes of ESIN between length stable and unstable PDFF. METHODS: We retrospectively reviewed patients undergoing ESIN treatment for isolated PDFF between 2016 and 2021. Exclusion criteria were (1) history of ipsilateral or contralateral lower extremity fractures, (2) highly comminuted or segmental fractures, (3) body weight >50 kg, and (4) comorbidities affecting bone quality, range of motion, or neurologic status. The patients were divided into 2 groups according to length stability. Clinical, radiographic, and pedobarographic data were then assessed to compare groups. RESULTS: Twenty-five patients were included (17 length stable and 8 length unstable PDFF) with a mean age of 73.6±17.8 months. There was no significant difference between groups in age, side of injury, body weight, follow-up duration, and nail-canal diameter ratio. Mean deformity in the fracture site in the early postoperative x-rays was not significantly different between groups (P=0.661). After a mean follow-up of 27.8±14.2 months (range, 12-67), there was no significant difference in mechanical axis deviation, distal femur joint orientation angle, or limb-length discrepancy in both groups. The pedobarographic assessment revealed that the length unstable group had a significantly higher external foot progression angle in the injured extremity (9.8°±6.9° vs. 1.3°±5.6°, P=0.031). However, the length stable group had no significant difference in the foot progression angle (4.9°±5° vs. 3°±4.3°, P=0.326). There was no significant difference in either group for other pedobarographic parameters. CONCLUSION: ESIN is a safe and effective option for length-unstable PDFF, yet attention should be paid to the rotational alignment. Although significant external rotation deformity occurs in length-unstable PDFF, it has no implications for the other pedobarographic parameters. LEVEL OF EVIDENCE: Level IV.

Fracture Patterns and Mortality in Osteopetrosis: A 7-year Retrospective Analysis from Türkiye's National Registry.

BACKGROUND: The aim of this study is to determine the demographic data, fracture treatment methods, and medical treatments of patients diagnosed with osteopetrosis in the national registry. METHODS: Patients with International Classification of Diseases (ICD)-10 code Q78.2 for osteopetrosis between January 1, 2016 and April 11, 2023 were retrospectively reviewed. Data on sex, age at time of diagnosis, fracture history, mortality, and use of medications were evaluated for all patients. In addition, open reduction and internal fixation, closed reduction and internal fixation, closed reduction and casting, and conservative treatment methods were noted. The number of patients requiring deformity surgery was determined. The incidence and prevalence of osteopetrosis were also calculated in this cross-sectional study. RESULTS: A total of 476 patients diagnosed with osteopetrosis were identified. The mean age at time of diagnosis of these patients was 5.79 ± 5.43 years. A total of 101 patients died. As the age at diagnosis decreased, the mortality rate of the patients increased with statistical significance ( P <0.001). A total of 192 fractures were seen in 121 osteopetrosis patients in this study. Femur fractures were most common among these patients with osteopetrosis. A history of fracture was statistically significantly less common in patients using a combination of vitamin D + calcium compared with patients not using such medication ( P <0.001). In this 7-year cross-sectional study, the incidence was found to be 1 in 416,000 and the prevalence was 0.00199% in the population under 18 years of age. CONCLUSION: Younger age at diagnosis is associated with higher mortality in patients with osteopetrosis. In addition, the combination of vitamin D and calcium were associated with lower fracture incidence. LEVEL OF EVIDENCE: Prognostic Level II.

Anatomic acetabular reconstruction with femoral head autograft for developmental dysplasia of the hip (DDH) with a minimum follow-up of 10 years.

BACKGROUND: DDH with coxarthrosis causes significant deformity and bone deficiency. Various reconstructive techniques have been proposed to treat developmental dysplasia of the hip. However, the existing literature has not yet reached a consensus on the best technique regarding long-term survival. OBJECTIVES: This study aims to evaluate the long-term survival of uncemented hydroxyapatite (HA) coated acetabular components augmented with a femoral head autograft. METHODS: We retrospectively reviewed the cases of 31 hips in 29 patients (24 female, 5 male, mean age 45.06 years) treated with HA-coated cementless components and femoral head autograft between 2000-2008 with a minimum follow-up of 10 years. Graft resorption, cup loosening and the anatomical hip centre were determined. Functional outcomes were calculated using the Harris Hip Scoring system. The survival of the acetabular component was evaluated using the Kaplan-Meier method. RESULTS: In 24 hips (77,4%), we reconstructed the hip centre anatomically. The remaining cups had variable deviations from the anatomical rotation centre. Only one patient required revision due to loosening. Survival analysis revealed 96.8% survival at 10 years. The mean Harris Hip Score was 39.23 preoperatively and 84.77 at final follow-up. There was no statistical correlation between revision and any of the measured parameters. DISCUSSION: Acetabular reconstruction with a femoral head autograft allows for anatomical cup positioning, early structural support and increases bone stock for future revisions. Although our prior cemented cup study showed that anatomical cup orientation is critical, this study demonstrated the absence of a correlation between implant failure and cup positioning, suggesting that HA-coated cementless cups are more stable and forgiving. CONCLUSIONS: HA-coated acetabular cups augmented with femoral head autograft provided long-term, reliable and durable cup fixation in dysplastic hips of young adults.

Magnetically Controlled Growing Rods Graduation: Deformity Control with High Complication Rate.

STUDY DESIGN: A multicenter retrospective review of consecutive series of patients. OBJECTIVE: Long-term experience with using the magnetically controlled growing rods (MCGR) to treat patients with deformity in the growing spine to the conclusion of treatment with posterior spine fusion. SUMMARY OF BACKGROUND DATA: MCGR treatment for growing spine gained popularity with paucity of long-term follow up data. We hypothesized that final fusion might be more effective in bringing additional correction of the spine deformity after treatment with MCGR than that reported after traditional growing rods (TGR) due to less scarring and auto-fusion. METHODS: Retrospective review of 47 patients with varied etiology, treated between 2011 and 2017 which graduated treatment were followed in five academic medical centers for average of 50 months (range, 10-88). RESULTS: The initial mean coronal deformity of 69.6° (95% CI 65-74) was corrected to 40° (95% CI 36-40) immediately after the MCGR implantation but progressed to 52.8° (95% CI 46-59) prior to the final surgery (P < 0.01). Nevertheless, thoracic spine growth (T1-T12 height) improved from 187.3 mm (95% CI 179-195) following index surgery to 208.9 mm (95% CI 199-218) prior to final fusion (P < 0.01). Significant correction and spinal length were obtained at final fusion, but metallosis was a frequent observation (47%, 22/47). The average growth rate was 0.5 mm/month (95% CI 0.3-0.6). The overall complication rate within our cohort was 66% (31/47) with 45% (21/47) of unplanned returns to the operating theater. 32% (15/47) of the patients had an implant related complication. Unplanned surgery was highly correlated with thoracic kyphosis greater than 40° (OR 5.42 95% CI 1.3-23). CONCLUSION: Treatment of growing spine deformities with MCGR provides adequate control of spine deformity it is comparable to previously published data about TGR. The overall high complications rate over time and specifically implant related complications.Level of Evidence: 4.

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.

Aponeurotic release of semimembranosus: A technical note to increase correction gained with hamstring lengthening surgery in cerebral palsy.

OBJECTIVE: The aim of this study was to determine the intraoperative corrective effect of the aponeurotic release of semimembranosus (SM) as a single procedure or an adjunct procedure to distal myotendinous release of semitendinosus (ST) and myofascial release of SM lengthening in the correction of knee flexion deformity in cerebral palsy (CP). METHODS: In this prospective study, 46 knees of 23 consecutive ambulatory patients (15 boys and 8 girls; mean age=8.33 years; age range=5-12 years) with spastic diplegic CP with a gross motor function classification system level (GMFCS) II or III were included. The patients were then divided into 2 groups. In group I, there were 10 patients (4 boys, 6 girls; mean age=8.6±2), and combined release of ST in the myotendinous junction and SM in the myofascial junction, followed by aponeurotic release of SM were carried out. In group II, there were 13 patients (2 girls, 11 boys; mean age=8±2.35), and aponeurotic release of SM was done first and followed by the combined release of ST in the distal myotendinous junction and the myofascial release of SM. Intraoperative popliteal angle (PA) measurements were recorded in each group. RESULTS: PA was reduced from 58.1°±7.6° (range=46°-75°) to 41.2°±8.8° (range=20°-54°) in group 1 and from 59.1°±11.3° (range=40°-87°) to 42.7°±10.8° (range=24°-64°) in group 2. No significant difference was observed between the groups in terms of reduction in PA (p=0.867). In group 1, adding the aponeurotic release of SM further reduced the PA to 31.7°± 8.5° (range=14°-47°) (p=0.002). In group 2, adding the myotendinous release of ST and myofascial release of SM further reduced the PA to 32.9°±7.2° (range=16°-44°) (p=0.004). There was no significant difference between the final PA values in the 2 groups (p=0.662). There was no difference in terms of early complications. CONCLUSION: Aponeurotic release of SM is equally effective to reduce the intraoperative PA with combined myotendinous release of ST and myofascial release of SM. Combining all the 3 procedures provides a better correction without forceful manipulation or lengthening of the lateral hamstrings during the correction of knee flexion deformity in CP.

Supracondylar Humerus Fractures in Older Children: Success of Closed Reduction and Percutaneous Pinning.

BACKGROUND: The incidence of supracondylar humerus (SCH) fracture declines and fracture types change as children grow. Optimal treatment method is unclear in older children. The aim of the study was to determine if fracture type and configuration of distal humerus fractures changes as patients approach skeletal maturity, and to assess the success of closed reduction and percutaneous pin (CRPP) in extra-articular SCH fractures in this transitional age group. METHODS: Inclusion criteria for this retrospective review were (1) distal humerus fractures with extension types 2 and 3, flexion type, T-type; (2) surgically managed, and (3) modified Sauvegrain score ≥1. Reviewed parameters included fracture type and configuration, grade of skeletal maturity, fixation technique, and loss of reduction. Primary analysis was to determine the distribution of fracture type and configuration with age or grade of skeletal maturity. Secondary analysis was used to determine the factors affecting treatment success of CRPP in extra-articular fractures. RESULTS: A total of 142 patients were included (58 males and 84 females). Fracture types revealed significant changes with increased age (P=0.031) and skeletal maturity grade (P<0.005). Skeletal maturity was a better predictor of changing fracture type than chronological age. T-type fractures were only seen in patients with modified Sauvegrain score ≥6 and flexion-type fractures were only seen in patients with modified Sauvegrain score ≤4. Loss of reduction rate after CRPP was 5%. The success of CRPP was not affected by age, sex, modified Sauvegrain score, fracture type, direction of displacement, coronal fracture pattern, number of pins or medial pin use. Fracture obliquity in the sagittal plane (P=0.05), suboptimal pin spread (P<0.01), and lack of bicolumnar fixation (P<0.01) were found as statistically significant factors associated with failed CRPP. CONCLUSION: The distribution of fracture type changed with increased age and skeletal maturity. CRPP of extra-articular fractures in older children is a reliable option regardless of the stage of skeletal maturity. Determinants of a good outcome include optimal pinning technique with adequate pin spread at the fracture site and bicolumnar fixation. LEVEL OF EVIDENCE: Level IV-retrospective 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.

Mid-term results of displaced acetabulum fractures surgically treated using anterior intra-pelvic approach (modified Stoppa).

BACKGROUND: This study aims to evaluate the radiological and clinical mid-term results of the patients with displaced acetabular fractures surgically treated with open reduction and internal fixation using an anterior intra-pelvic approach (AIP). METHODS: In this study, we retrospectively reviewed 12 patients with displaced acetabular fractures treated surgically via the AIP approach. Patients were analyzed for Letournel's acetabular fracture classification, associated injuries, time to surgery, additional surgical procedures needed, perioperative and postoperative complications, radiologic and functional results. RESULTS: Of the 12 patients, the male/female ratio was 1/2; the mean age was 40.5±16.2 (16-64) years. The mean follow-up time was 59.8±32.2 (12-124) months. Seven patients had both column fractures, three patients had anterior column + posterior hemitransverse fractures, one patient had transverse + posterior wall and one patient had anterior column fracture. The mean time to surgery was 6.6±4.4 (2-16) days. The mean intraoperative blood transfusion was 830 (300-2000) ml. Intra-operative and post-operative complications were noted in eight patients. The mean Merle d'Aubigné and Postel score was 14.5±2.7 (10-18). Six patients with an anatomical reduction of the fracture showed excellent/good functional and radiologic outcomes. Three patients with a non-anatomic reduction developed post-traumatic arthrosis that was treated with total hip arthroplasty. CONCLUSION: AIP approach provides a satisfactory exposure for the surgical treatment of displaced anterior wall/column and both column acetabular fractures. Clinical outcome is directly related to the reduction quality. Patients with poor reduction are most likely to develop mid-term complications, such as hip joint arthrosis.

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.

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.

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.

Pedicle Subtraction Osteotomy Versus Multiple Posterior Column Osteotomies in Severe and Rigid Neuromuscular Scoliosis.

STUDY DESIGN: Retrospective analysis. OBJECTIVE: The aim of this study is to compare the safety and efficacy of the apical pedicle subtraction osteotomy (PSO) technique with multiple posterior column osteotomies (PCOs) in nonambulatory patients with severe, rigid neuromuscular scoliosis. SUMMARY OF BACKGROUND DATA: Neuromuscular scoliosis frequently causes intolerance to sitting due to pelvic obliquity, trunk decompensation, and associated back and rib impingement pain which diminish the patient's functional capacity. In the case of rigid curves, spinal osteotomy techniques are occasionally required for effective correction. METHODS: We retrospectively reviewed our patients with severe and rigid neuromuscular scoliosis with associated pelvic obliquity who were treated with posterior instrumented fusion extending to pelvis with more than 1-year postoperative follow-up. We compared radiological and clinical results of PSO and multiple PCO techniques in severe rigid neuromuscular scoliosis with pelvic obliquity of more than 15° in traction radiograph under general anesthesia. Hospital records were also reviewed for operative time, intraoperative blood loss, amount of blood transfusion, duration of hospital stay, and complications. RESULTS: There were 12 patients in the PSO group and 10 patients in the PCO group. There was no significant difference between groups in terms of major curve magnitude, sagittal parameters or pelvic obliquity. Although not statistically significant, PSO technique did trend toward better scoliosis correction (post-op Cobb angle 56.1° vs. 66.7° [P = 0.415]). PSO technique provided a significantly better correction in pelvic obliquity (59% vs. 84%) (P = 0.001). There was no significant difference in average intraoperative blood loss, transfusion, and operative times including anesthesia time, hospital stay, or complications. CONCLUSION: PSO may be an option in correction of severe and rigid neuromuscular scoliosis. It provides better correction of pelvic obliquity without increasing operative time, need for transfusion, or duration of hospitalization as compared to multiple apical PCO technique. Traction radiograph under general anesthesia is a valuable tool in surgical decision making. LEVEL OF EVIDENCE: 4.

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.

Health-Related Quality of Life in Early-Onset Scoliosis Patients Treated Surgically: EOSQ Scores in Traditional Growing Rod Versus Magnetically Controlled Growing Rods.

STUDY DESIGN: Cross-sectional study. OBJECTIVE: To compare quality of life and caregiver burden in traditional growing rod (TGR) and magnetic controlled growing rods (MCGR) patients. SUMMARY OF BACKGROUND DATA: MCGR decrease surgical sessions associated with treatment of early onset scoliosis (EOS), hoping to minimize the burdens seen with repetitive invasive surgeries in TGR treatment. Although the clinical indications for these treatments have largely been agreed upon, there is a lack of understanding of their impact on patients' and families' quality of life. METHODS: Inclusion criteria: ≤10 years of age at index procedure, major curve ≥30°, no previous spine surgery, minimum 1-year postoperative follow-up. The previously validated 24-item early onset scoliosis questionnaire (EOSQ-24) was utilized to assess quality of life. Statistic methods were applied to compare domain scores between TGR and MCGR patients. RESULTS: Forty-four children with EOS were enrolled; 25 TGR and 19 MCGR. Groups were similar in sex and age at index surgery. Age at time of questionnaire and mean length of follow-up were significantly different; patients were older (14.0 vs. 8.8 yr) and had longer follow-up (101.3 vs. 34.3 mo) in TGR (P < 0.01). Deformity correction and complication rates were similar between groups. At the time of questionnaire, scores of economic burden and overall satisfaction in MCGR were significantly superior to those in TGR by univariate analysis. When controlled for duration of follow-up, some domain scores trended towards statistical significance, some remained stable, and others regressed to non-significance. CONCLUSION: Health related quality of life data reveal superior outcomes in overall satisfaction and financial burden domains in the MCGR group. However, the positive effects of MCGR decrease when controlled for length of follow up, indicating that the MCGR is not yet a magic fix-all, and that the TGR remains an option in the treatment of EOS. LEVEL OF EVIDENCE: 3.

Titanium mesh cage as an alternative reconstruction method for epiphyseal-sparing tumour resections in children.

In this study, we introduced an alternative reconstruction option for epiphysis-sparing tumour resection in children. Eight patients with a malignant tumour in the diaphysis or metaphysis-diaphysis junction of a long bone underwent epiphysis-sparing intercalary resection. Reconstruction was performed using a titanium mesh cage filled with impacted cancellous bone allograft and autograft. A plate and screws were used to supplement the fixation. At the last follow-up, union was achieved in seven patients. Limb-length discrepancy occurred in three patients. Functional scores revealed a good functional outcome. This technique may be an alternative method for epiphyseal-sparing tumour resections in children.

Paper #44: Pull-Out of the Upper Thoracic Pedicle Screws Can Cause Spinal Canal Encroachment in Growing Rod Treatment.

The aim of this study is to report the prevalence of spinal canal encroachment of pedicle screw in proximal anchor of GR construct and determine risk factors using a single-center cohort.