Safety and efficacy of growth-friendly instrumentation for early-onset scoliosis in patients with spinal muscular atrophy type 1 in the disease-modifying treatment era

Purpose: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy. Methods: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits. Results: Twenty-eight patients (mean follow-up: 16 months (range 2–41)) were included. The mean age at surgery was 60 months (range 29–96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded. Conclusion: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2

Cerebral Palsy And Spinal Deformities

Cerebral palsy is a common static encephalopathy and can present as multiple musculoskeletal problems. Increased peripheral muscle tone causes joint contractures and decreased functional capacity. The risk for scoliosis increases parallel with the severity of musculoskeletal involvement. Scoliosis adversely affects the functional capacity, daily care, and nutrition in disabled children. Conservative treatments including physical therapy, bracing, and botulinum toxin injections do not prevent the progression of scoliosis in most of the patients and surgical treatment becomes mandatory. With the use of pedicle screws, three-plane fixation is possible, making posterior instrumentation and fusion effective in correction of severe curves and obviating anterior surgery.WoSScopu

Testing the excavator performance (using Topcon 3d Excavator X63 System) especially for navigation and earthwork

Construction sites commonly utilize bulldozers, wheel loaders, excavators, scrapers, and graders. Among these, excavators are versatile hydraulic heavy-duty equipment operated by humans. They are employed for various tasks like digging, levelling the ground, transporting and dumping loads, as well as providing straight traction. However, certain hazardous environments, such as nuclear disasters or earthquakes, are not suitable for human on-site work. To enhance productivity, accuracy, and profitability in excavation projects, the adoption of 3D machine control is recommended. The Topcon 3D Excavator X63 System offers advanced and precise GNSS positioning technology, coupled with Hidromek with Assist and an intuitive software interface, to significantly improve excavation operations. In this study, the accuracy of the coordinates of the route followed by the Excavator was checked by using RTK GNSS method by using P1 reference point. While the differences obtained in horizontal coordinates are 2–2.5 cm and 4–6 cm in vertical coordinates. In addition, excavation calculations of the earthwork area were performed and checked with the number of bucket of the excavator. The differences obtained from the earthwork were calculated as 0.8 cubic meters for each bucket

Surgical management of post-SCIWORA spinal deformities in children

Background: Patients with spinal cord injury without radiographic abnormality (SCIWORA) are prone to develop spinal deformities. The purpose of this study is to report on the clinical and radiological features of post-SCIWORA spinal deformities. Methods: Four patients with SCIWORA and spinal deformities requiring surgery were analyzed clinically and radiographically. Results: All four SCIWORA patients developed progressive neuromuscular scoliosis. There were 2 males and 2 females. The mean age at spinal cord injury was 3.9 years (range 6 months to 7 years). Spinal deformity was first noticed at a mean of 17 months after their initial injury (range 9 months to 2 years), and surgical intervention was performed at a mean of 6.5 years following their injury (range 4-11 years). The mean preoperative curve was 54° (range 50-62°). The mean postoperative curve was 9.5° (range 5-16°). The level of injury was T5 in two patients, and T10 and L2 in one patient each. All but the lumbar level injury patient had complete paraplegia. One patient with complete injury (T10) and another with incomplete injury (L2) improved neurologically and were able to walk with the aid of orthoses and crutches. Pelvic obliquity improved in all patients following spinal reconstruction surgery, and none of the patients required additional surgery for spinal deformity. The mean postoperative follow-up was 51.75 months (range 24-93 months). Conclusions: Long scoliotic curves extending to the pelvis should be expected in SCIWORA. Early intervention may prevent severe deformities. Modern instrumentation techniques employing pedicle screws provided satisfactory correction, improved hip subluxation, and did not adversely affect the ambulatory ability or functional level of the patients in this series. © 2010 EPOS

Convex Instrumented Hemiepiphysiodesis With Concave Distraction: A Preliminary Report

The convex growth arrest (CGA) procedure has been well accepted for treatment of congenital scoliosis as it is a simpler procedure with successful results. However, unpredictability of curve behavior, slow and usually inadequate correction, and necessity of anterior surgery for completeness of the epiphysiodesis are its shortcomings. In a preliminary study we asked whether a modification of the CGA procedure using convex instrumented hemiepiphysiodesis with concave distraction would correct the coronal plane Cobb angles and would correct or maintain sagittal plane local and global kyphosis angles. We also identified complications. We retrospectively reviewed five female patients who underwent the modified procedure. Their mean age at the index operation was 40 months (range, 17-55 months). The patients underwent concave distractions every 6 months. The magnitude of the convex instrumented and concave distracted curves and sagittal plane parameters were determined on the preoperative and most recent followup radiographs. Minimum followup was 26 months (mean, 34 months; range, 26-40 months). In the coronal plane, the preoperative magnitude of the convex instrumented congenital curve averaged 48A degrees. It was corrected to 36A degrees (25%) postoperatively and was further improved to 27A degrees (44%) at the latest followup. For the distracted segment, the mean preoperative curve was 35A degrees, corrected to 16A degrees postoperatively and to 8A degrees at the latest followup, for an average correction of 77%. Sagittal plane alignment was minimally affected from the procedure. In four of the five patients we identified partial pullout of screws for the concave distraction; these were revised at the time of planned lengthening. This procedure may obviate the need for multiple osteotomies and long thoracic fusions in young children with long sweeping thoracic deformities involving multiple anomalous vertebrae. Implant-related complications on the concave side may be avoided using paired pedicle screws at the proximal and distal anchor sites. Level IV, therapeutic study. See the guidelines online for a complete description of level of evidence.Wo

Assessment of Quality of Life in Children After Successful Treatment of Hip Dysplasia As Compared with Normal Controls

Objective: To document the quality of life of children who have been treated for developmental dysplasia of the hip (DDH) within the first 3 years of life successfully after a minimum follow-up period of 2 years, and compare their results with healthy age-matched children. Methods: Thirty patients with DDH treated between 1998 and 2002 in Ankara Numune Education and Research Hospital, Ankara, Turkey and 19 age-matched healthy controls were included in this study with the approval of the ethical committee. The mean follow up period was 56.1 months. Each child's parents answered the Child Health Questionnaire-Parent Form 50 (CHQ-PF50) after giving informed consent. The answers were evaluated, and scores were calculated and results were analyzed. Results: There were no significant differences in 14 health-related quality of life concepts, according to the CHQ instrument's outcomes, between the healthy controls and the patients with DDH who were successfully treated within the first 3 years of life. Only the physical functioning concept was found to be lower for the DDH patients (Mann Whitney U test, p = 0.036). Conclusion: Although children who were successfully treated for DDH may have impairment in physical functioning after a certain period of follow-up, there was no significant impairment in physical and psychosocial summary scores, in comparison with their age-matched controls.Wo

Long-Segment Posterior Instrumentation and Fusion with Freeze-Dried Allograft in Congenital Scoliosis

Objective: The aim of this study was to evaluate the effectiveness of long-segment posterior instrumentation and allograft application in obtaining fusion in congenital scoliosis. Methods: Twenty-one patients with congenital scoliosis who were treated with long-segment posterior instrumentation (>6 levels) and freeze-dried allograft and followed up for more than 24 months were reviewed. Six patients were excluded from the study due to anterior procedures. Fifteen patients with congenital scoliosis (13 females, 2 males; mean age: 12.2 +/- 3,rears, range: 7-17 years) were retrospectively reviewed. Mean follow-up time was 30.9 +/- 9.4 (range: 24 to 48) months. Six patients had laminectomy either due to previous posterior surgeries or to address intraspinal pathologies during the posterior fusion procedure. Preoperative, postoperative and final follow-up anteroposterior and lateral spine X-rays were reviewed. Fusion was graded according to the classification reported by Bridwell et al. Results: Two patients were graded as 'no fusion' (pseudarthrosis), four patients as 'probably fused', and nine patients as 'definitely fused'. The major curve was corrected from an average of 68 +/- 18.6 to 39.3 +/- 12.2 degrees (p<0.001). Mean correction lost in the major curve was an average of 4.5 +/- 5.2 degrees in the latest follow-up. There was significant correction in the compensatory curve (preoperative 37.9 +/- 13.2 degrees, postoperative 20.2 +/- 6.6 degrees; p=0.001). Preoperative and postoperative global thoracic kyphosis were 39.5 +/- 13.3 and 32.3 +/- 7.9 degrees, respectively (p=0.018). Preoperative and postoperative global lumbar lordosis was 36.3 +/- 7.4 and 36.1 +/- 8.9 degrees, respectively (p=0.883). Successful fusion was detected in %86.7 of patients in the final follow-up. Conclusion: The usage of allograft alone to achieve fusion increases the rates of pseudarthrosis while additional anterior procedure decreases the pseudarthrosis rate in patients with congenital scoliosis that require long-segment posterior instrumentation. Further studies should be performed to assess the efficacy of the usage of polysegment pedicle screw instrumentationWoSScopu

Safety and Efficacy of Instrumented Convex Growth Arrest in Treatment of Congenital Scoliosis

WOS: 000335698200007PubMed ID: 24045587Background: Anterior and posterior convex hemiepiphysiodesis is a widely used surgical alternative in the treatment of congenital scoliosis. This procedure has the disadvantage of the need for both anterior and posterior approaches. Furthermore, outcomes may be unpredictable. Posterior convex growth arrest (CGA) with pedicle screws at each segment on the convex side may obviate the need for anterior surgery and provides more predictable outcomes. This study retrospectively evaluates the safety and efficacy of instrumented posterior CGA in congenital scoliosis. Methods: Patients who had posterior CGA with convex pedicle screw instrumentation for congenital scoliosis were evaluated retrospectively. Thirteen patients (6 male, 7 female) were included in the study. Preoperative, early postoperative, and last follow-up standing posteroanterior and lateral x-rays were evaluated. Cobb angles were recorded for the instrumented segment (main curve). Global thoracic kyphosis was measured between T2 and T12 on sagittal plane. These values were compared preoperatively, postoperatively, and at last follow-up. The T1-S1 vertical height and the height between the concave side pedicles of the upper and lower end vertebra of the main curve was also determined and recorded as the concave height. Results: The average follow-up was 56.1 +/- 10 months (range, 36 to 74 mo) and the average age of the patients at the time of operation was 64.5 +/- 30.1 months (range, 15 to 108 mo). All patients were Risser zero at the time of surgery. The average curve magnitude was 49 +/- 10.9 degrees (range, 34 to 68 degrees) preoperatively, 38.3 +/- 9.7 degrees (range, 28 to 58 degrees) early postoperatively, and 33.5 +/- 12.4 degrees (16 to 52 degrees) at last follow-up. There was a significant difference between the preoperative and early postoperative main curve Cobb angle measurements (P=0.001). The average concave height was 94.2 +/- 20.2 mm in the early postoperative period and 104.7 +/- 21.7 mm at last follow-up (P=0.003). The average T1-S1 height was 292.1 +/- 67.1 mm in the early postoperative period and 363.9 +/- 94.5 mm at last follow-up (P=0.005). There was at least >= 5 degrees improvement in 9 of the 12 patients in the follow-up period after the index procedure. In 3 patients, the curve did not change and the correction was maintained. Curve progression was observed in 1 patient due to a technical error. There were no wound infections or instrumentation failures during follow-up. Conclusions: Instrumented CGA can safely be used in long sweeping curves of immature spines. Using this technique; thoracotomy, anterior procedure, and 2-stage surgery can be avoided. Moreover, it guarantees some degree of correction in all patients because of the instrumentation effect, eliminating the unpredictable nature of classic CGA. Level of Evidence: Therapeutic level IV study