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.

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.

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.

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.

Reliability and Validity of the Adapted Turkish Version of the Early-onset Scoliosis-24-Item Questionnaire (EOSQ-24).

INTRODUCTION: Early-onset scoliosis (EOS) can have negative effects on the developing thorax, lungs, and quality of life in general. Children with EOS can face various health problems and require recurring hospitalization and surgeries. Radiographic parameters are insufficient to evaluate the severity and efficacy of treatment in EOS. Early-onset Scoliosis Questionnaire (EOSQ)-24 questionnaire is a new instrument developed for this specific age group. To date, reliability of this questionnaire has not yet been interrogated in wide patient groups from different cultures. The aim of this study was to evaluate the validity and reliability of culturally adapted Turkish version of the EOSQ-24. METHODS: Forward translation and back translation of the English version of the EOSQ-24 was done, and all steps for cross-cultural adaptation process were performed properly by an expert committee. Turkish version of the EOSQ-24 and Child Health Questionnaire-Parent Form-50 (CHQ-PF-50) were applied to 61 (24 male, 37 female) EOS patients. The average age of these patients was 9.1 ± 3.1 years (0.4 to 14.3 y), and 50 of them had undergone surgical treatment. Data quality was assessed by mean, median, percentage of missing data, and extent of ceiling and floor effects. Reliability was assessed by internal consistency using Cronbach's α and item-total correlations. The construct validity was evaluated by comparing the results of the EOSQ-24 with the Turkish version of the CHQ-PF-50. Subgroup analyses were applied for sex, diagnosis, treated/untreated, mobilization ability, and complications. RESULTS: The item response to the EOSQ-24 was high with a small number of missing answers (1.6% to 3.3%). Of the 24 items, 22 were evenly distributed. This resulted in a floor effect in 0% to 21.7% of patients, and a ceiling effect in 1.6% to 68.3%. The calculated Cronbach's α for the 24-item scale was 0.909, indicating excellent reliability. Construct validity showed that high correlations between the EOSQ and the CHQ by means of similar domains. Correlation coefficient was between 0.348 and 0.688 (P=0.0001). Subgroup analyses also showed significant difference in treated/untreated patients (P=0.032) and mobilization ability (P=0.001). DISCUSSION: The Turkish adaptation of the EOSQ-24 exhibits favorable psychometric properties and excellent reliability, validating its use in this population.

Serial derotational casting in congenital scoliosis as a time-buying strategy.

BACKGROUND: Serial casting is an effective treatment modality in early-onset idiopathic scoliosis; however, the role of this method in congenital scoliosis is not well studied. METHODS: A total of 11 patients with progressive congenital scoliosis were treated with serial cast application. Age at initial cast application, magnitudes of the congenital, compensatory and sagittal deformities, coronal balance, T1 to T12 height, number of casts and time-in cast per patient, subsequent surgical interventions, and complications were evaluated. RESULTS: Mean age at the first cast application was 40 months, and the average number of cast changes was 6.2 per patient. There were no major complications. The average precasting curve magnitude was 70.7 degrees (range, 44 to 88 degrees) and was significantly reduced to 55.1 degrees (range, 16 to 78 degrees) at the latest follow-up (P=0.005). The average precasting compensatory curve was 55.8 degrees (range, 38 to 72 degrees) and was significantly reduced to 39.8 degrees (range, 23 to 62 degrees) at the latest follow-up (P=0.017). Average T1 to 12 height increased from 12.8 cm at post-first cast to a 14.6 cm at the latest follow-up (P=0.04). Average time in cast was 26.3 months (range, 13 to 49 mo). During the treatment period, none of the patients required surgery for curve progression. CONCLUSIONS: Serial derotational casting is a safe and effective time-buying strategy to delay the surgical interventions in congenital deformities in the short-term follow-up. LEVEL OF EVIDENCE: Level IV, case series.