Neuromuscular Scoliosis Research Articles

Elevated hospital floor-based HDU (POPUP-HDU): a new safe alternative to PICU for high-risk neuromuscular and syndromic children undergoing scoliosis surgery.

Children undergoing either posterior spinal fusion (PSF) or index insertion of growing rods for neuromuscular or genetic/syndromic scoliosis may require post-operative care on the paediatric intensive care unit (PICU). Demands on this limited resource result in frequent bed shortage related cancellations. In response, an ad-hoc or 'pop-up' ward-based high-dependency unit (POPUP-HDU) was developed. This converts a ward bed to POP-HDUbed for the required time. This study assesses the safety and efficacy of postoperative management that utilises POPUP-HDU as an alternative to a PICU bed. Retrospective review of 111 consecutive children undergoing posterior surgery for scoliosis between June 2016 and April 2023. The inclusion criteria included a diagnosis of genetic/syndromic or neuromuscular scoliosis; PSF or primary insertion of distraction-based growth rods and requirement for postoperative care in a PICU. We excluded those children that were mandated to go to PICU post-operatively for any reason by the anaesthetic team. 49 patients (mean age 13.0years) were managed on PICU, and 62 (mean age 11.4years) on POPUP-HDU. The groups were matched with respect to body weight, curve magnitude, operative duration, type of fusion procedure performed, the presence of cardiac malformations, the use of home breathing support, the number of operated levels, pelvic instrumentation and intraoperative blood loss. 8 patients in the PICU, and 16 in the POP-HDU groups were readmitted back to PICU following step-down to the hospital ward (p = 0.27). The median PICU length of stay was 1day in the PICU group and less than a day in POPUP-HDU (for those that needed to be subsequently admitted to PICU). The median total length of hospital stay was 10days in the PICU group, and 8days in POPUP-HDU (p < 0.05). 14 patients developed medical complications in the PICU group, compared to 19 in POPUP-HDU. There were no bedshortage cancellations in POPUP-HDU, compared to 23 in PICU. For children with neuromuscular, genetic or syndromic scoliosis undergoing PSF or growth rods that are not deemed suitable for immediate ward-level post-operative care, POPUP-HDU provided a safe alternative to PICU for appropriate patients and was associated with shorter hospital stay and fewer cancellations for lack of PICU beds. Therapeutic Level III.

Vertebral Body Morphology in Neuromuscular Scoliosis with Spastic Quadriplegic Cerebral Palsy

Background/Objectives: The distorted vertebral body has been studied in scoliosis; however, there is little knowledge about the difference between neuromuscular and idiopathic scoliosis. This study aimed to investigate the vertebral body morphology in patients with spastic quadriplegic cerebral palsy and scoliosis (CP scoliosis) and compare them with those of apex- and Cobb angle-matched patients with adolescent idiopathic scoliosis (AIS). Methods: Thirty-four patients with CP scoliosis and thirty-two patients with AIS were included. The pedicle diameter, chord length, and vertebral body rotation were evaluated at one level above the apex, one level below the apex, and at the apex using a reconstructed computed tomography scan. The apex of the curve and Cobb angle were too diverse between patients with CP scoliosis or AIS. Eighteen patients were matched in each group according to the apex and Cobb angle (within 5-degree differences) of the major curve, and compared between matched groups (mCPscoliosis vs. mAIS). Results: In the comparison of the apex and Cobb angle-matched groups, there was no statistical difference in the Cobb angle between mCPscoliosis (80.7 ± 13.8 degrees) and mAIS (78.6 ± 13.6 degrees, p = 0.426), and the vertebral body rotation (25.4 ± 15.4° in mCPscoliosis vs. 24.4 ± 6.5° in mAIS, p = 0.594). There was no difference in the pedicle diameters of either the convex (3.6 ± 1.1 mm in mCPscoliosis vs. 3.3 ± 1.2 mm in mAIS, p = 0.24) or concave side (3.1 ± 1.2 mm in mCPscoliosis vs. 2.7 ± 1.6 mm in mAIS, p = 0.127). However, the patients in the mCPscoliosis group were younger (12.7 ± 2.5 years vs. 14.6 ± 2.4 years, p = 0.001), and the chord length was shorter on the convex (38.0 ± 5.0 mm vs. 40.4 ± 4.9 mm, p = 0.025) and concave (37.7 ± 5.2 mm vs. 40.3 ± 4.7 mm, p = 0.014) sides compared with those of the mAIS group. Conclusions: With a similar apex and Cobb angle, the vertebral body rotation and pedicle diameter in patients with CP scoliosis were comparable to those with AIS; however, the chord length was shorter in CP scoliosis. For the selection of the pedicle screw in CP scoliosis, the length of the pedicle screw should be more considered than the diameter.

Long-term results of single-stage posterior hemivertebra resection and short segment fusion using pedicle screws fixation in thoracolumbar congenital early-onset scoliosis: an 8.97-year average follow-up study

BackgroundThis study aims to evaluate the long-term outcomes of one-stage posterior hemivertebra resection and short segment fusion with pedicle screw fixation in children with thoracolumbar congenital early-onset scoliosis (TLCEOS). It specifically investigates the durability of spinal correction and the incidence of complications over an average follow-up of 8.97 years.MethodsA retrospective review was conducted on 32 patients treated at a tertiary scoliosis referral center from April 2003 to December 2019. Inclusion criteria were thoracolumbar hemivertebra (T11-L1), treatment via posterior hemivertebra resection and short segment fusion (≤ 5 segments), age under 10 years at surgery, and a follow-up of at least 5 years. Exclusions included idiopathic, syndromic, or neuromuscular scoliosis. Data on demographics, surgical procedures, and radiographic outcomes were collected, and clinical outcomes were evaluated using the Scoliosis Research Society-22 (SRS-22) questionnaire. Statistical analysis was performed using SPSS and R software.ResultsThe study included 32 patients (22 males, 10 females) with a mean age of 5.00 ± 2.42 years at surgery and an average follow-up of 8.97 ± 2.81 years. On average, 3.47 ± 1.11 segments were fused. The main curve corrected from 38.64° ± 14.12° preoperatively to 9.06° ± 7.29° postoperatively, with a final correction rate of 61%. Significant improvements were observed in the SRS-22 domains of Appearance and Satisfaction with Treatment. There were 17 complications, including neurological, pulmonary, and mechanical issues, with some cases requiring revision surgery.ConclusionsOne-stage posterior hemivertebra resection and short segment fusion with pedicle screw fixation effectively correct TLCEOS long-term, offering significant improvements in patient appearance and satisfaction, with a manageable complication profile.

Changes in Diaphragm Intrusion and Thoracic Dimensions After Posterior Spinal Fusion in Patients With Neuromuscular Scoliosis.

Cerebral palsy (CP) can cause scoliosis with large thoracolumbar or lumbar curves. Such curves may impair pulmonary function by causing the abdomen and diaphragm to encroach on the thorax. Our purpose was to investigate changes in diaphragm position and other thoracic radiographic measurements at 2 years after posterior spinal fusion (PSF). Retrospective review of data from 56 pediatric patients (Gross Motor Function Classification System >3) who underwent PSF for CP-related (neuromuscular) scoliosis at one US academic hospital from 2010 to 2018. In this study, we used radiographs taken preoperatively and 2 years after PSF to measure lung volume, diaphragm intrusion index (DII), diaphragm vertebral level (DVL), space available for the lung (SAL), and T1-S1 height. Lung volume had increased by a mean 902cm3 (range, -735 to 2697cm3) at 2-year follow-up. DII improved from a mean (and SD) of 61%±12% to 71%±11% on the left side and 58%±14% to 68%±11% on the right (P<0.001). DVL increased caudally by a mean 1.2 vertebral levels bilaterally, with a mean postoperative position between T8 and T9. Lung space became more symmetrical as the SAL increased from 0.76 to 0.91 (P<0.001). T1-S1 height increased by a mean 7.5±4.3cm. These findings suggest a new way to understand changes in thoracic volume and redistribution of thoracic and lumbar balance when correcting the collapsing spinal deformity in CP. A more caudal postoperative diaphragm position with less diaphragm intrusion into the thorax may reflect an improved length-tension configuration, which could in turn produce greater diaphragmatic strength and endurance. Level III.

Comparison of two modeling approaches for the identification of predictors of complications in children with cerebral palsy following spine surgery

BackgroundChildren with non-ambulatory cerebral palsy (CP) frequently develop progressive neuromuscular scoliosis and require surgical intervention. Due to their comorbidities, they are at high risk for developing peri- and post-operative complications. The objectives of this study were to compare stepwise and LASSO variable selection techniques for consistency in identifying predictors when modelling these post-operative complications and to identify potential predictors of respiratory complications and infections following spine surgery among children with CP.MethodsIn this retrospective cohort study, a large administrative claims database was queried to identify children who met the following criteria: 1) ≤ 25 years old, 2) diagnosis of CP, 3) underwent surgery during the study period, 4) had ≥ 12-months pre-operative, and 5) ≥ 3-months post-operative continuous health plan enrollment. Outcome measures included the development of a post-operative respiratory complication (e.g., pneumonia, aspiration pneumonia, atelectasis, pleural effusion, pneumothorax, pulmonary edema) or an infection (e.g., surgical site infection, urinary tract infection, meningitis, peritonitis, sepsis, or septicemia) within 3 months of surgery. Codes were used to identify CP, surgical procedures, medical comorbidities and the development of post-operative respiratory complications and infections. Two approaches to variable selection, stepwise and LASSO, were compared to determine which potential predictors of respiratory complications and infection development would be identified using each approach.ResultsThe sample included 220 children. During the 3-month follow-up, 21.8% (n = 48) developed a respiratory complication and 12.7% (n = 28) developed an infection. The prevalence of 11 variables including age, sex and 9 comorbidities were initially considered to be potential predictors based on the intended outcome of interest. Model discrimination utilizing LASSO for variable selection was slightly improved over the stepwise regression approach. LASSO resulted in retention of additional comorbidities that may have meaningful associations to consider for future studies, including gastrointestinal issues, bladder dysfunction, epilepsy, anemia and coagulation deficiency.ConclusionsPotential predictors of the development of post-operative complications were identified in this study and while identified predictors were similar using stepwise and LASSO regression approaches, model discrimination was slightly improved with LASSO. Findings will be used to inform future research processes determining which variables to consider for developing risk prediction models.

Spine to pelvis “T-Construct” using magnetic controlled growing rods in non-walkers neuromuscular early-onset scoliosis: a preliminary study

BackgroundsMagnetic controlled growing rods (MCGRs) have been proven to be effective in controlling early onset neuromuscular scoliosis but no study has evaluated the combination with a sacro-bi-iliac construct. The aim of our study is to report surgical management of early onset non-walkers neuromuscular scoliosis correction using MCGRs associated with a sacro-bi-iliac “T-construct” and its mid-term outcomes. Our hypothesis was that this set-up provided well correction of the pelvic obliquity and that this correction was maintained over time. MethodsA retrospective single-center study was conducted including all consecutive neuromuscular early onset scoliosis who underwent spinopelvic fixation using “T-construct” with two MCGRS. Four millimeters lengthening was performed every 4 months during outpatient clinics sessions. All children had a low-dose biplanar stereoradiography in EOS-Chair at pre/postoperative phase, each outpatient clinic appointment and last follow-up. ResultsEighteen patients were included and 17 analyzed at the last follow-up. The mean age at surgery was 9.5 (range from 5 to 12 years), the mean follow-up was 4,7 years (range from 2.5 to 6.6 years) and 8 patients had a Risser stage above four. The global complication rate was 35% (N = 6/17 patients) including three medical and three mechanical complications related to “T-construct”, while the reoperation rate was 18% (N = 2 patients for wound debridement and one for iterative pelvic fixation). Cobb angle and pelvic obliquity were significantly improved by surgery (mean correction was 33.2 ° (55%) and 11 ° (77%) respectively; p < 0.001). At the last follow-up, we noted a loss of frontal Cobb angle correction (p < 0.01) whereas we did not observe any significant loss of pelvic obliquity (p > 0.9). ConclusionsAlthough the global complication rate was 35% (half of which are mechanical complications), the treatment combining pelvic T-construct and MCGRs provides satisfactory correction of pelvic obliquity correction, good maintenance in the medium term and may be a procedure to consider for the surgical treatment of early onset neuromuscular scoliosis. Level of evidenceIV; Retrospective cohort prognostic study

Caregiver Values and Preferences Related to Surgical Decision-making for Children with Medical Complexity

To uncover the values and preferences of the caregivers for children with medical complexity (CMC), using the test case of surgical treatment decision-making for pediatric neuromuscular scoliosis (NMS) that will inform the future development of a decision support tool in this population. We conducted a qualitative study of semi-structured interviews of English- and Spanish-speaking caregivers of children with NMS from two geographically distinct children's hospitals. We used purposive sampling of language and treatment options selected to capture diverse experiences. Analysis was based on grounded theory with synthesized caregiver values and preferences themes. From 47 participants, we completed 41 interviews (9 in Spanish). Caregivers had a mean age of 43.2 years, were mostly White (66%), and had children with a mean age of 15.6. 64% chose surgery. The following values and preferences were important to many caregivers: reducing scoliosis-related pain, minimizing mobility limitations to optimize socio-emotional quality of life, limiting the impact of comorbidities on overall quality of life, information provided by peer support, the uncertainty of outcomes due to underlying comorbidities, and the uncertainty related to the anticipated progression of their child's scoliosis curve. Caregivers experienced immense uncertainty related to treatment outcomes due to their child's comorbidities. Caregivers of CMC may benefit from decision support that includes both values clarification exercises to help caregivers identify what of the many possible values and preferences are important to them and novel methods to communicate uncertainty in the care of CMC.

A retrospective study of anesthesia management in patients operated for neuromuscular scoliosis

Objective: Scoliosis frequently develops as a complication of neuromuscular diseases, often progressing and necessitating surgical intervention. Although complications can arise in spinal fusion surgery for all types of scoliosis, they are more frequent during and after the procedure in cases of neuromuscular scoliosis. This retrospective study aims to explore the preoperative characteristics, anesthesia protocols, as well as intra- and postoperative complications and associated conditions in patients who underwent surgery for neuromuscular scoliosis at our hospital.Material and Methods: After receiving approval from the ethics committee of our hospital, this study conducted a retrospective review of patient files from individuals who underwent surgery for neuromuscular scoliosis at Ankara Training and Research Hospital between 2008 and 2012. A total of 26 patient files were analyzed. Patient parameters including age (years), gender (female (F), male (M)), weight (kg), presence of neuromuscular disease, concomitant cardiovascular and respiratory conditions, as well as other systemic anomalies and diseases, spirometry findings (FEV1, FVC, FEV1/FVC), nutritional status (total protein, albumin), pre-operative hemoglobin (Hg) and hematocrit (Htc) levels, Cobb angle index, angle direction, type of surgical approach (anterior or posterior), muscle relaxants utilized, additional dosage requirements, operation duration, intraoperative bleeding volume (ml), transfusion volume (ml), and intraoperative complications were documented. Results: As the Cobb angle increased, several factors were affected: the duration of the operation was extended (p &lt; 0.05), there was an increase in blood loss (p = 0.012), and more blood transfusions were required (p = 0.32). Furthermore, there was a correlation between increasing age and the amount of blood transfused (p = 0.035). Conclusion: It has been concluded that a comprehensive preoperative assessment is crucial, as it can offer valuable insights into anesthesia management both before and after surgery for scoliosis. Therefore, conducting a detailed preoperative evaluation is essential for patients undergoing these procedures

Topical vancomycin powder for the prevention of surgical site infections in spinal deformity surgery: a systematic review and meta-analysis.

To assess the effectiveness and safety of topical vancomycin powder (VP) in preventing surgical site infections (SSIs) in spinal deformity surgeries. A literature search was conducted on Web of Science, PubMed, and Cochrane Library databases for comparative studies of VP in spinal deformity surgeries published before February 2024. Two reviewers independently screened eligible articles based on the inclusion and exclusion criteria, assessed study quality, and extracted data. Data analysis was performed using Review Manager 5.4 software. Of all 143 papers screened, a meta-analysis was conducted on 10 articles, which included a total of 8,166 surgeries. The results of the meta-analysis indicated that the incidence of deep SSI in VP group was 0.28 times that in non-VP group (p < 0.001). In the subgroup analysis, VP treatment significantly reduced the risk of deep SSI in both adult spinal deformity (ASD) (RR 0.40, 95% CI 0.21-0.77, p = 0.006) and pediatric scoliosis (PS) (RR 0.25, 95% CI 0.16-0.38, p < 0.001) surgeries. However, this effect was not observed in neuromuscular scoliosis (NMS) patients (RR 0.66, 95% CI 0.26-1.66, p = 0.38). Bacterial culture results indicated that VP treatment significantly reduced polymicrobial infections (p = 0.007) and gram-positive infections (p = 0.001). From the literature available at present, VP was associated with reduced deep SSIs rates in spinal deformity patients. However, particular attention should be paid to the lack of the effectiveness of VP in NMS patients. The current literature did not report local cytotoxicity or renal toxicity related to VP in spinal deformity patients.

"Ultralow-dose" CT Without Sedation in Pediatric Patients With Neuromuscular Scoliosis.

Children with neuromuscular scoliosis undergoing scoliosis surgery face substantial rates of complications. To mitigate surgical risks such as blood loss in pediatric patients with neuromuscular scoliosis, this study focuses on enabling instrumentation planning for their abnormal vertebral and pelvic anatomy and osteopenia. This study assessed the feasibility of an "ultralow-dose" CT (ULD CT) protocol without sedation in pediatric patients with neuromuscular scoliosis who often have comorbid movement disorders. Our prospective quality improvement study aims: (1) to determine if ULD CT without sedation is feasible in this patient group; (2) to quantify the radiation dose from ULD CT and compare it with preoperative spine radiographs (XR); and (3) to assess if ULD CT allows accurate anatomical assessment and intraoperative navigation given the prevalence of movement disorders. Children with neuromuscular scoliosis underwent spine XR and ULD CT scans. Chart reviews assessed disease etiology and comorbidities. Radiation dose was quantified through Monte-Carlo simulations giving dose indices and effective dose, with statistical analysis done using a paired student's t-test (α=0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation. Fourteen patients (5 males, 9 females, average age 14±3y) participated. One patient needed sedation due to autism spectrum disorder and global developmental delay. The radiation dose for spine XR was 0.5±0.2mSv, and ULD CT was 0.6±0.1mSv. There was no statistically significant difference in radiation doses between methods. All ULD CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2 Alar-Iliac screw orientation, and intraoperative navigation. ULD CT without sedation is feasible for children with neuromuscular scoliosis. Radiation doses were comparable to standard radiographs. ULD CT provided accurate anatomical assessments and supported intraoperative navigation, proving beneficial despite movement disorders in these patients. Level 2-Development of diagnostic criteria on basis of consecutive patients (with universally applied reference widely accepted standard).

In-hospital post-operative fever after posterior spinal fusion for neuromuscular scoliosis in non-ambulatory cerebral palsy patients: is there value for clinical workup for an isolated fever?

Children with neuromuscular scoliosis (NMS) resultant to cerebral palsy (CP) are at a heightened risk for complications following surgical treatment. These children have a reported 22-64% rate of post-operative fever development, and additional fever workup has been shown to have limited clinical utility. However, this has yet to be investigated in the setting of an accelerated discharge (AD) pathway. A retrospective review of children with non-ambulatory CP treated at 2 centers with posterior spinal fusion (PSF) for NMS was performed. One institution uses a standardized AD post-operative pathway for NMS patients, whereas the second institution had no standard pathway. A post-operative fever was defined as temperature > 38.5°C. Target outcome variables included the development of a fever as well as re-admission within 90days of surgery. A total of 122 non-ambulatory children were identified (82% GMFCS V, mean 14.3 ± 3.4years at surgery). A post-operative fever was documented in 75.4% of patients (N = 92) and all additional culture studies reported negative results. Children admitted to the PICU were more likely to undergo a fever workup (P < 0.001) and more likely to receive additional or extended antibiotic therapy (P < 0.001). Children treated at the AD pathway had a significantly lower rate of PICU admission (P < 0.001). Post-operative PICU admission was associated with a post-operative fever (49.5% vs 25%, P = 0.03). Non-ambulatory CP children with NMS undergoing PSF have a 75.4% rate of developing early post-operative fevers. Reflexive fever work-ups provided limited clinical utility while increasing the hospital length of stay and potentially exposing patients to antibiotic-related complications.

Unveiling the adverse events of Nusinersen in spinal muscular atrophy management based on FAERS database

This study aims to collect and analyze adverse event (AE) reports related to Nusinersen from the FAERS database. The study employed a combination of signal quantification techniques, including the Reporting Odds Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Multi-item Gamma Poisson Shrinker (MGPS), to enhance the accuracy of signal detection and reduce the risk of false positives or negatives. Between the first quarter of 2017 and the third quarter of 2023, the FAERS database collected a total of 11,485,105 drug AE reports, of which 5772 were related to Nusinersen. Through signal mining analysis, 218 preferred term (PT) signals involving 27 system organ classes (SOCs) were identified. The study discovered AEs related to metabolism and nutrition disorders, psychiatric disorders, and cardiac disorders SOCs, which were not mentioned in the product information. Additionally, complications directly related to the intrathecal administration of Nusinersen, such as increased CSF pressure, positive CSF red blood cell count, and AEs related to the method of drug use, such as neuromuscular scoliosis and cerebrospinal fluid reservoir placement, were highlighted. Notably, AEs related to renal function abnormalities, such as abnormal Urine protein/creatinine ratio and protein urine presence, showed higher frequency and signal strength. The findings of this study emphasize the importance of comprehensive safety monitoring in the clinical application of Nusinersen. These results are significant for guiding future clinical practices, improving disease management strategies, and developing safer treatment protocols.

Can We Screen for Limb Length Discrepancy on Spinal Radiographs of Patients With Adolescent Idiopathic Scoliosis?

The aim of this study was to determine whether height differences in the levels of the iliac crests and femoral heads on erect spinal radiographs can be used as indirect measurements for the screening and surveillance of limb length discrepancy (LLD) in patients with scoliosis. Whole body posteroanterior (PA) and lateral erect radiographs of patients with adolescent idiopathic scoliosis (AIS) were retrospectively reviewed. Patients with congenital, syndromic, and neuromuscular scoliosis were excluded. A direct measurement of each limb was taken from the highest point of the femoral head to the middle of the tibial plafond; any difference between the sides was recorded as the LLD. In addition, the PACS Software tool was used to measure femoral head height difference (FHHD) and iliac crest height difference (ICHD). Pearson's correlation, linear regression, and Bland-Altman plots were used to determine the relationships between LLD and FHHD, and LLD and ICHD. Radiographs of 141 patients (92 women, 49 men) with an average age of 12.0±2.65 years were analyzed. A strong correlation (r=0.730, P<0.001) was found between LLD and FHHD; the correlation between LLD and ICHD was weaker (r=0.585, P<0.001). The Bland-Altman analysis showed good agreements of LLD with FHHD and ICHD. Linear regression analysis predicted an LLD of ≤10mm based on an FHHD of ≤11.5mm or an ICHD of ≤15.3mm. FHHD and ICHD on spinal PA radiographs can be used for the screening and monitoring of LLD in patients with AIS with FHHD being the preferred indirect measurement. These measurements are readily learned and quick to perform. The FHHD and ICHD can be measured on any erect scoliosis PA radiograph. Therefore, these proxy measurements can be used to screen and monitor for LLD in patients with AIS. Level III.

Frequency and management of rod fractures following minimally invasive bipolar fusionless surgery in neuromuscular scoliosis patients

IntroductionNeuromuscular scoliosis (NMS) is associated with an abnormal muscle tone. Traditional conservative treatments, with the historical practice of early posterior fusion, have proven ineffective. Recently, growth-sparing techniques have gained traction owing to their ability to maximize trunk height. However, these techniques have a substantial risk of complications, particularly rod breakage, with reported incidence rates ranging from 15 % to 42 %. The objective of this study was to conduct a descriptive analysis of NMS patients who experienced rod breakage following the minimally invasive fusionless surgery (MIFS) technique. MethodsThis was a single-center, retrospective study that included all NMS patients who underwent surgery between January 2015 and January 2021 and subsequently presented with rod breakage after MIFS. The MIFS technique is based on proximal fixation with double hook claws made of pedicular and -sus laminar hooks and pelvic fixation with iliosacral screws. ResultsThe mean follow-up was 5.2 ± 2.2 years. The mean dominant etiology of NMS was cerebral palsy (67 %). Of the 217 patients who underwent surgery, 15 (6.9 %) developed rod breakage. Rod breakage occurred 2.7 ± 1.3 years after the initial surgery. Four cases of rod fracture recurrence were reported in ambulatory patients with dystonia or hyperactivity. ConclusionCompared with other fusionless techniques, the minimally invasive bipolar technique appears promising for patients with NMS, with a lower rate of rod breakage. We recommend the use of a four-rod construct for ambulatory patients or for those with dystonia or hyperactivity.

Rib-on-Pelvis Deformity and Reported Pain in Neuromuscular Early-Onset Scoliosis.

Neuromuscular early-onset scoliosis (N-EOS) often presents with a long sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the pelvis, termed rib-on-pelvis deformity (ROP). The goal of this study is to evaluate whether ROP is associated with reported pain and other health-related quality of life (HRQOL) measures. We hypothesize that ROP is associated with increased pain and negative HRQOL. A multicenter international registry was queried for all nonambulatory patients with N-EOS from 2012 to 2022. Both surgical and nonsurgical patients were included. ROP was classified as a binary radiographic assessment of preoperative (surgical patients) and most recent follow-up (nonsurgical patients) upright radiographs. Reported pain and other HRQOL measures were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). Patients with nonupright radiographs or EOSQ-24 questionnaires and corresponding radiographs >4 months apart were excluded. Totally, 225 patients (8.4±3.1y, 55% female) were included. The median major curve was 63.3 (IQR: 40.6 to 81.2) degrees and median pelvic obliquity was 15.5 degrees (IQR: 8.8 to 26.4). Eighty-three patients (37%) had ROP. ROP was associated with both frequency (P<0.001) and severity (P<0.001) of pain. ROP was associated with worse general health (P=0.01), increased difficulty with vocalization (P=0.02), increased frequency of shortness of breath (P=0.002), and increased difficulty sitting upright (P=0.04). Regarding overall EOSQ-24 domains, ROP was associated with worse general health, pain/discomfort, pulmonary function, and physical function (P<0.01). In a subanalysis of 76 patients who underwent surgical intervention with at least 2 years of follow-up, patients with preoperative ROP experienced significantly greater improvements in both frequency (P=0.004) and severity (P=0.001) of pain than the patients without preoperative ROP at 2 years postoperatively. The overall incidence of ROP in N-EOS is about 37%. ROP is associated with greater pain and worse HRQOL through the EOSQ-24 questionnaire. Furthermore, these patients experienced a greater reduction in pain after surgery. Clinicians and parents must be aware that ROP is possibly a pain generator, but responds positively to surgical intervention. Level III.

Complications and Health-Related Quality of Life in Children with Various Etiologies of Early-Onset Scoliosis Treated with Magnetically Controlled Growing Rods-A Multicenter Study.

Background: Early-onset scoliosis (EOS) refers to spinal deformities that develop and are diagnosed before the age of 10. The most important goals of the surgical treatment of EOS are to stop the progression of curvature, achieve the best possible correction, preserve motion, and facilitate spinal growth. The objectives of this multicenter study were to analyze the risk of complications among patients with EOS treated using magnetically controlled growing rods (MCGRs) and assess the patients' and their parents' quality of life after diagnosis and treatment with a minimum two-year follow-up. Methods: Patients given an ineffective nonoperative treatment qualified for surgery with MCGRs. This study involved 161 patients (90 females and 71 males) who were classified according to the etiology of curvature. The intraoperative and postoperative complications and those that occurred during the continuation of treatment with MCGRs were recorded and analyzed. The 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) was used to evaluate the patients' quality of life and satisfaction with the treatment. Results: Implant-related complications requiring instrumentation revision were recorded in 26% of the patients. Medical complications occurred in 45% of the population. The EOSQ-24 revealed a significant improvement in the average scores during the follow-up. Conclusions: The treatment of early-onset scoliosis with MCGRs carries 66% risks of incurring medical and mechanical complications, the latter 26% of patients requiring revision procedures. Children with neuromuscular scoliosis, females, and with curvature greater than 90 degrees are at a higher risk of developing complications. Limiting the number of elective surgeries necessitated to prolong the instrumentation and treatment process for patients with MCGRs can greatly enhance their quality of life and satisfaction throughout the follow-up period.

Pelvic Fixation for Non-Ambulatory Patients with Neuromuscular Scoliosis Treated with Magnetically Controlled Growing Rods-A 4-Year Comparison of Two Different Fixation Methods.

Background/Objectives: This study aims to analyze the efficacy and safety of the two pelvic fixation systems, S-hooks (SH) and lumbar-sacral-alar-iliac (SAI) screws, when used in association with magnetically controlled growing rods (MCGRs) in non-ambulatory children with severe neuromuscular scoliosis (NMS). Methods: Forty-one patients were retrospectively examined and subdivided corresponding to ilium hook fixation or SAI screws. The major curve correction (%) and pelvic obliquity (PO) correction (%) were assessed utilizing scoliosis plain film radiographs over time. Complications and unplanned return to the operating room (UPROR) were recorded. Patient-specific pre- and postoperative values were included in a backward stepwise regression model to assess UPROR. Results: Mean age at index intervention was 9.4 years. Preoperative main curve was 81° and PO was 22°. 21 and 20 patients were categorized into the SH and SAI subgroups, respectively. Initial curve correction was significantly better in the SAI subgroup (63 vs. 42% in the SH, p = 0.045), while PO correction was equally good. Curve and PO correction were maintained throughout the follow-up period of 55 months. UPROR rate was 38% in the SH subgroup, and 5% in the SAI subgroup (p = 0.010). Regression analysis identified postoperative curve correction as predictive value for UPROR (p = 0.006). Conclusions: SAI screw fixation has a low UPROR rate and achieves superior curve correction. S-hooks are a viable option to correct PO and NMS in children with high operative risk to reduce operative time, but revision surgery is not uncommon.

How Many Imaging Studies Do Patients With Neuromuscular Early Onset Scoliosis (EOS) Receive?

Children with neuromuscular early onset scoliosis (EOS) receive numerous radiographic studies both from orthopaedic and other specialties. Ionizing radiation doses delivered by computed tomography (CT) are reportedly 100 times higher than conventional radiography. The purpose of this study was to evaluate the number of radiographic studies ordered for neuromuscular EOS patients during their care. Retrospective review at a tertiary children's hospital from January 2010 to June 2021 included all patients with neuromuscular EOS followed by an orthopaedic specialist for a minimum of 3 years. Patients were excluded if the majority of their nonorthopaedic care was provided by outside institutions. Eighteen patients met inclusion criteria with mean follow up of 6.4±2.3 years. A total of 1312 plain radiographs and 35 CT scans were performed. Of the plain radiographs, 34.7% were ordered by orthopaedic providers and 65.3% (857/1312) were ordered by other providers. Of the CT scans, 4 were ordered by orthopaedic providers, while 88.5% (21/35) were ordered by other providers. An average of 74.7 (range: 29 to 124) radiographs and 1.9 (range: 0 to 9) CT scans ordered over the course of each patient's treatment for an average of 13.0±6.0 radiographs and 0.3 CT scans per year. With an average of 75 radiographs and 1.9 CT scans performed per patient, consideration for steps to limit exposure to ionizing radiation should be made a particularly high priority in this unique subset of patients. This requires interdisciplinary coordination as 65% of the radiographs and over 80% of the CT scans were ordered by nonorthopaedic providers. Level III.

Placement of ilio-sacral screws in fusionless technique for pediatric neuromuscular scoliosis utilizing planning software, in conjunction with intraoperative navigation, results in a safer optimal screw: a CT-based study.

The insertion of ilio-sacral (IS) screws for distal anchoring in the instrumentation of pediatric neuromuscular scoliosis (NS) presents a significant challenge, often leading to elevated rates of complications. Utilizing computed tomography (CT) navigation and preoperative planning technology is proposed as a potential solution to mitigate these challenges. This study aims to assess the precision of IS screw placement through CT-graphic measurements, both with and without preoperative planning, followed by navigated IS screw insertion, in pediatric neuromuscular scoliosis. Thirty-two treated patients were grouped based on surgical procedure: planned (P): 19 patients (n=38 screws) and non-planned (NP): 13 patients (n=26 screws). All screw placements (P and NP) took place under CT navigation. IS screw trajectories of P-group were drawn preoperatively on CT images with the cranial trajectory planning program and fused with the intraoperative CT images. There are several important anatomical structures that should be avoided when placing the IS screw (L5 root, spinal canal, L5S1 facet, SI joint, neurovascular structures anteriorly to the sacrum, S1 root in the S1 foramen and the intestines). Each trajectory was evaluated based on seven radiographical parameters whom we have enlisted partially based on the essentials of a good trajectory described by Miladi et al. (1: Ilium; 2: SI joint; 3: Promontorium; 4: Sacral plate; 5: Anterior sacral cortex; 6: S1 foramen; 7: Spinal canal). An independent sample T test was executed to compare both groups. The trajectories in the P group showed a significantly (P<0.05) higher overall similarity and optimality (12.1±2.1 vs 9.1±2.2 points) compared to the non-planned trajectory. Preoperative planning and navigated placement of IS screws on fusion images with intraoperative CT, results in a better trajectory of the ilio-sacral screws.

Development of Consensus-Based Best Practice Guidelines for the Peri- and Post-operative Care of Pediatric Patients with Spinal Deformity and Programmable Implanted Devices

Study Design. Modified Delphi consensus study. Objective. To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery. Summary of Background Data. Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery. Guidelines for monitoring and interrogating these devices during the peri-operative period are not available. Methods. A panel was assembled consisting of 25 experts (i.e., spinal deformity surgeons, neurosurgeons, neuro-electrophysiologists, cardiologists, and otolaryngologists). Initial postulates were based on literature review and results from a prior survey. Postulates addressed the following IPDs: vagal nerve stimulators (VNS), programmable ventriculo-peritoneal shunts (VPS), intrathecal baclofen pumps (ITBP), cardiac pacemakers and implantable cardioverter-defibrillators (ICD), deep brain stimulators (DBS), and cochlear implants. Cardiologist and otolaryngologists participants responded only to postulates on cardiac pacemakers or cochlear implants, respectively. Consensus was defined as ≥80% agreement, items that did not reach consensus were revised and included in subsequent rounds. A total of three survey rounds and one virtual meeting were conducted. Results. Consensus was reached on 39 total postulates across six IPD types. Postulates addressed general spine surgery considerations, use of intraoperative monitoring and cautery, use of magnetically-controlled growing rods (MCGRs), and use of an external remote controller to lengthen MCGRs. Across IPD types, consensus for the final postulates ranged from 94.4-100%. Overall, experts agreed that MCGRs can be surgically inserted and lengthened in patients with a variety of IPDs and provided guidance for the use of intraoperative monitoring and cautery, which varied between IPD types. Conclusion. Spinal deformity correction surgery often benefits from the use of intraoperative monitoring, monopolar and bipolar cautery, and MCGRs. Final postulates from this study can inform the peri- and post-operative practices of spinal deformity surgeons who treat patients with both scoliosis and IPDs. Level of Evidence. V- Expert opinion