Neuromuscular Early-onset Scoliosis Research Articles

Incidence of Parental Requests to Discontinue Growth-Friendly Surgical Lengthening for Early Onset Scoliosis.

The STOP questionnaire was developed to document reasons for discontinuation of growth-friendly (GF) treatment in early onset scoliosis (EOS). This study investigated the incidence of parental request (PR) on the STOP questionnaire and compared clinical information and Early Onset Scoliosis 24-Item Questionnaire (EOSQ-24) scores of PR patients with those whose parents did not request discontinuation (non-parent request [NPR]). An international pediatric spine registry was queried for EOS patients with STOP questionnaires completed by their surgeon. Age at discontinuation, sex, and EOS etiology were recorded. GF device, number of surgical procedures, complications, STOP questionnaire reasons for discontinuation, and definitive treatment were recorded. EOSQ-24 scores and clinical information in the PR cohort were compared with the NPR cohort. Data for 1326 patients were analyzed. PR was listed on the STOP questionnaires of 46 (3.5%) patients, completed at a mean age of 12 years (SD, 3.2 years). There were no statistical differences in number of procedures or complications when comparing the PR cohort with the NPR cohort. PR patients more frequently had neuromuscular EOS (P=.002), more frequently were treated with magnetically controlled growing rods (33% vs 14%, P=.036), and more frequently were observed after GF discontinuation (P=.628). EOSQ-24 scores for the PR cohort were significantly lower in most domains except pain/discomfort. For 3.5% of the EOS patients, PR was listed on the STOP questionnaire. They frequently had neuromuscular EOS and frequently were treated with magnetically controlled growing rods. Additionally, these patients had statistically lower EOSQ-24 scores across most domains. [Orthopedics. 20XX;4X(X):XXX-XXX.].

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.

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.

Do Pedicle Screws That Terminate in the Costovertebral Joint Compromise Proximal Anchor Fixation in a Dual Growing Rod Construct?

Growing rods (GRs) are used to treat early-onset scoliosis (EOS) recalcitrant to bracing and casting. Proximal anchor pullout, a known complication of GR constructs, can result in spinal cord injury if pedicle screw anchors are placed with a lateral-to-medial trajectory. To mitigate this risk, a more straightforward and potentially safer trajectory may result in screws that terminate within the costovertebral joint (CVJ). We asked, how often does CVJ placement occur and does this technique increase the rate of failure in GR constructs? We retrospectively reviewed 35 patients with EOS treated with dual GR with >2-year follow-up excluding patients with skeletal dysplasia or history of previous posterior instrumentation. Patient demographics, radiographic parameters, and implant constructs were assessed. Of patients meeting the inclusion criteria, 18/35 (51%) were females with an average age of 7.96 (range: 4.0 to 15.2) years at surgery with a follow-up of 3.7 (range: 2.0 to 7.7) years. Five (14%) patients had idiopathic EOS, 5 (14%) had congenital EOS, 12 (34%) had neuromuscular EOS, 10 (29%) had syndromic scoliosis, and 3 (9%) had another etiology for EOS. Among 195 proximal pedicle screws placed, 19 (10%) terminated within the CVJ, and 13 patients (37%) had at least 1 CVJ screw. Two patients (6%) experienced unilateral proximal pullout. In both patients, the anchors on the affected side included 1 of 2 screws within the CVJ. Both patients had constructs that included 2 screws on the side that pulled out and 3 screws on the side that did not. None of the remaining 17 CVJ screws led to implant failure. Pedicle screw placement within the CVJ is common and does not appear to significantly contribute to proximal screw pullout; however, it may contribute to unilateral implant failure in constructs employing only 2 proximal screws, where 1 of those 2 screws terminates within the CVJ. Construct modifications should be considered in this scenario. Level III.

Degree of Postoperative Curve Correction Decreases Risks of Postoperative Pneumonia in Patients Undergoing Both Fusion and Growth-friendly Surgical Treatment of Neuromuscular Scoliosis.

Due to a combination of poor respiratory muscle control and mechanical lung compression secondary to spine and chest wall deformities, patients with neuromuscular (NM) early-onset scoliosis (EOS) are at a high risk for pulmonary complications including pneumonia. The purpose of this study is to examine the effect of surgical intervention on the prevalence and risk of postoperative pneumonia in patients with NM EOS. In this retrospective cohort study, pediatric (18 y old and below) patients with NM EOS undergoing index fusion or growth-friendly instrumentation from 2000 to 2018 were identified. Patients were then categorized into 2 groups: those with ≥50% curve correction and those with <50% curve correction of the coronal deformity at the first postoperative visit. The primary outcome of interest was postoperative pneumonia occurring between 3 weeks and 2 years postoperatively. Manual chart review was supplemented with phone call surveys to ensure all occurrences of preoperative/postoperative pneumonia (ie, in-institution and out-of-institution visits) were accounted for. A total of 35 patients (31% female, age at surgery: 10.3±4.3 y) with NM EOS met inclusion criteria. Twenty-three (66%) patients experienced at least 1 case of preoperative pneumonia. Twenty-six (74%) patients had ≥50% and 9 (26%) patients had <50% immediate postoperative curve correction. In total, 12 (34%) patients experienced at least 1 case of postoperative pneumonia (7 in-institution, 5 out-of-institution). Seven (27%) patients with ≥50% curve correction versus 5 (56%) with <50% curve correction experienced postoperative pneumonia. Relative risk regression demonstrated that patients with <50% curve correction had increased risk of postoperative pneumonia by 2.1 times compared with patients with ≥50% curve correction (95% confidence interval: 0.9; 4.9, P =0.099). The prevalence of preoperative and postoperative pneumonia is high in patients with NM EOS. This study presents preliminary evidence suggesting that percent curve correction is associated with the occurrence of postoperative pneumonia in patients with NM EOS undergoing surgical correction.

Mortality in Neuromuscular Early Onset Scoliosis Following Spinal Deformity Surgery.

The purpose of this study was to report mortality and associated risk factors in neuromuscular early onset scoliosis following spinal deformity surgery. This is a multicenter retrospective cohort study of patients with cerebral palsy (CP), spinal muscular atrophy, myelodysplasia, muscular dystrophy, or myopathy undergoing index spine surgery from 1994 to 2020. Mortality risk was calculated up to 10 years postoperatively. Proportional hazard modeling was utilized to investigate associations between risk factors and mortality rate. A total of 808 patients [mean age 7.7 y; 439 (54.3%) female] were identified. Postoperative 30-day, 90-day, and 120-day mortality was 0%, 0.001%, and 0.01%, respectively. 1-year, 2-year, 5-year, and 10-year mortality was 0.5%, 1.1%, 5.4%, and 17.4%, respectively. Factors associated with increased mortality rate: CP diagnosis [hazard ratio (HR): 3.14, 95% confidence interval (CI): 1.71; 5.79, P<0.001]; nonambulatory status (HR: 3.01, 95% CI: 1.06; 8.5, P=0.04)]; need for respiratory assistance (HR: 2.17, 95% CI: 1.00; 4.69, P=0.05). In neuromuscular patients with early onset scoliosis, mortality risk at 10 years following spine surgery was 17.4%. As mortality was 1.1% at 2 years, premature death was unlikely a direct result of spine surgery. Diagnosis (CP) and markers of disease severity (nonambulatory status, respiratory assistance) were associated with increased mortality rate. Prognostic level II.

Baclofen Pump Use: Complications After Growth-friendly Instrumentation for Early-onset Scoliosis.

Patients with early-onset scoliosis (EOS) and spasticity may receive treatment with an intrathecal baclofen pump. We assessed how baclofen pumps are associated with the odds of complications and secondary interventions after growth-friendly (GF) spine surgery for EOS and analyzed infectious complications within the pump cohort. Using a prospectively maintained, international multicenter database, we studied patients with neuromuscular EOS with baclofen pumps who underwent GF spine surgery from 2002 through 2019 (n=25). Baclofen pumps were implanted before GF instrumentation in 18 patients, during in 2 patients, and after in 5 patients. Patients with existing pumps at initial GF spine surgery were matched 1:3 with 54 patients (control group) without pumps according to treatment center, year of surgery, diagnosis, surgery type, and preoperative curve magnitude. Univariate analysis and multivariate logistic regression were performed to compare complications and secondary interventions between the 2 cohorts. Patients with baclofen pumps had 4.8 times the odds [95% confidence interval (CI): 1.5-16] of experiencing any complication within 1 year after initial GF spine surgery compared with controls. During mean follow-up of 6.9±4.3 years, they had 4.7 times the odds (95% CI: 1.3-16) of deep surgical site infection and 5.6 times the odds (95% CI: 1.2-26) of spinal rod removal after any complication. Differences in rates of mechanical complication, such as rod migration and breakage, were nonsignificant between the 2 groups. For the 9 patients (50%) with pumps who experienced infections, the most common microorganisms were Staphylococcus aureus (4 patients) and Pseudomonas aeruginosa (2). The pump/catheter was revised or removed, in addition to antibiotic therapy or surgical irrigation and debridement, in 2 patients. Among patients with neuromuscular EOS, those with baclofen pumps are much more likely to experience complications within 1 year after GF spine surgery. They are also more likely to have deep surgical site infections, with S. aureus and P. aeruginosa being the most common causative organisms, and to require spinal rod removal. Level III-retrospective comparative study.

Growth-friendly surgery results in more growth but a higher complication rate and unplanned returns to the operating room compared to single fusion in neuromuscular early-onset scoliosis: a multicenter retrospective cohort study

Compare radiographic outcomes, complications, and QoL in neuromuscular early-onset scoliosis (EOS) patients treated with single posterior spinal fusion (PSF) versus growth-friendly surgery and definitive fusion (GFDF). In a retrospective cohort study, children with neuromuscular EOS, age 8-11years at index surgery with PSF or GF devices, with minimum 2-year follow-up after final fusion were identified from a multicenter database. 16 PSF and 43 GFDF patients were analyzed. Demographics were similar except PSF patients were older at index surgery and had shorter follow-up. PSF patients had greater percentage major curve correction (62% vs 38%, p = 0.001) and smaller major curve at final follow-up (23° vs 40°, p = 0.005). The GFDF group underwent over five times more surgeries (8.7 vs 1.6, p = 0.0001). Four PSF patients (25%) experienced ten complications, resulting in five unplanned returns to the operating room (UPROR) in three patients (19%). 36 GFDF patients (84%) experienced 83 complications, resulting in 45 UPRORs in 24 patients (56%). Poisson regression adjusted for age showed that the GFDF group had more complications (p = 0.001) and UPRORs (p = 0.01). Although the GFDF patients had smaller preoperative T1-T12 and T1-S1 lengths, these were similar to the PSF patients at final follow-up, indicating that the GFDF patients had greater spinal growth. PSF patients had better postoperative EOSQ-24 Financial Impact and Family Burden scores. While there was a difference in age at index surgery, PSF may be more effective than GFDF at controlling neuromuscular EOS. GFDF patients achieved more spinal growth but eight times more complications and nine times more UPRORs.

One-Way Self-Expanding Rod in Neuromuscular Scoliosis

Background:Fusionless techniques for the treatment of neuromuscular early-onset scoliosis (EOS) are increasingly used to preserve spinal and thoracic growth and to postpone posterior spinal fusion (PSF). These techniques have greatly improved thanks to magnetically controlled growing rods, which allow the avoidance of repeated surgery. However, the surgery-related complication rate remains high. The objective of the current study was to report the preliminary outcomes of 21 patients with neuromuscular EOS who were treated with a 1-way self-expanding rod (OWSER). This device was designed to avoid repeated surgery and preserve spinal and thoracic growth thanks to its free rod sliding.Methods:Patients with neuromuscular EOS who underwent OWSER fixation were prospectively reviewed; follow-up was a minimum of 3 years. The instrumentation relies on a bipolar construct from T1 to the sacrum, with proximal fixation by double thoracic hook-claws and distal fixation by iliosacral screws. The device comprises a rod with a notched part sliding in 1 direction inside a domino. Changes in Cobb angle, pelvic obliquity, thoracic kyphosis, lumbar lordosis, T1-S1 and T1-T12 length, space available for the lung, and chest width were assessed. Complications were reviewed.Results:The mean age at surgery was 10.5 years. The mean follow-up was 3.9 years. The mean pelvic obliquity improved from 20° preoperatively to 8° postoperatively and to 6° at the latest follow-up. The mean Cobb angle improved from 66° preoperatively to 38° postoperatively and to 32° at the latest follow-up. The mean preoperative kyphosis was reduced from 41° to 26° at the latest follow-up (p = 0.14). The mean lordosis was 34° preoperatively and 38° at the latest follow-up. The mean growth per month was 0.8 mm for the T1-T12 segment and 1.5 mm for T1-S1. The global complication rate was 38% (2 surgical site infections, 3 cases of lack of rod expansion, 1 case of pyelonephritis, and 2 central venous catheter-related infections). No PSF had been performed at the latest follow-up.Conclusions:Use of the OWSER with a minimally invasive bipolar technique for neuromuscular EOS provided satisfactory correction of spinal and pelvic deformities at 3 years of follow-up. A longer follow-up is required.Level of Evidence:Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Mid-term results of a modified self-growing rod technique for the treatment of early-onset scoliosis.

To report the mid-term results of a modified self-growing rod (SGR) technique for the treatment of idiopathic and neuromuscular early-onset scoliosis (EOS). We carried out a retrospective analysis of 16 consecutive patients with EOS treated with an SGR construct at a single hospital between September 2008 and December 2014. General demographics and deformity variables (i.e. major Cobb angle, T1 to T12 length, T1 to S1 length, pelvic obliquity, shoulder obliquity, and C7 plumb line) were recorded preoperatively, and postoperatively at yearly follow-up. Complications and revision procedures were also recorded. Only patients with a minimum follow-up of five years after surgery were included. A total of 16 patients were included. Six patients had an idiopathic EOS while ten patients had a neuromuscular or syndromic EOS (seven spinal muscular atrophy (SMA) and three with cerebral palsy or a syndrome). Their mean ages at surgery were 7.1 years (SD 2.2) and 13.3 years (SD 2.6) respectively at final follow-up. The mean preoperative Cobb angle of the major curve was 66.1° (SD 8.5°) and had improved to 25.5° (SD 9.9°) at final follow-up. The T1 to S1 length increased from 289.7 mm (SD 24.9) before surgery to 330.6 mm (SD 30.4) immediately after surgery. The mean T1 to S1 and T1 to T12 growth after surgery were 64.1 mm (SD 19.9) and 47.4 mm (SD 18.8), respectively, thus accounting for a mean T1 to S1 and T1 to T12 spinal growth after surgery of 10.5 mm/year (SD 3.7) and 7.8 mm/year (SD 3.3), respectively. A total of six patients (five idiopathic EOS, one cerebral palsy EOS) had broken rods during their growth spurt but were uneventfully revised with a fusion procedure. No other complications were noted. Our data show that SGR is a safe and effective technique for the treatment of EOS in nonambulatory hypotonic patients with a neuromuscular condition. Significant spinal growth can be expected after surgery and is comparable to other published techniques for EOS. While satisfactory correction of the deformity can be achieved and maintained with this technique, a high rate of rod breakage was seen in patients with an idiopathic or cerebral palsy EOS. Cite this article: Bone Joint J 2020;102-B(11):1560-1566.

Shilla Growth Guidance Compared With Magnetically Controlled Growing Rods in the Treatment of Neuromuscular and Syndromic Early-onset Scoliosis.

Retrospective review of consecutive series of patients treated at two institutions. The aim of this study was to compare the health-related quality of life (HRQoL) and surgical outcomes of Shilla growth guidance and magnetically controlled growing rod (MCGR) treatment in patients with syndromic and neuromuscular early-onset scoliosis (EOS). Knowledge of the outcomes of Shilla instrumentation is limited. We identified 13 children treated with Shilla and 18 children treated with MCGR with syndromic or neuromuscular EOS (major curve ≥45 degree) before the age of 10 years with minimum 2-year follow-up. Outcome parameters included clinical data, radiographic as well as HRQoL outcomes (EOSQ-24 questionnaire). Mean preoperative major curves were 64 degree (range, 45-108 degree) in the Shilla group and 58 degree (range, 45-85 degree) in the MCGR group (P = 0.151). At final follow-up, mean major curves were 31 degree (range, 9.4-54 degree ) and 30 degree (range, 16-53 degree), respectively (P = 0.392). The mean major curve correction was 45% in the Shilla group and 48% in the MCGR group during the follow-up (P = 0.383). Spinal (T1-S1) and T1-T12 growth were significantly better (P = 0.006 and 0.042) in the MCGR than in the Shilla group during the distraction period. At final follow-up, 11 (85%) children in the Shilla group and 17 (94%) in the MCGR group had achieved T1-T12 length of ≥18 cm (P = 0.202). There were significantly more surgical procedures in the MCGR group (mean 2.6 vs. 1.4, P = 0.034) with no difference in the number of complications (P = 0.768). EOSQ24 domains were similar at final follow-up. Shilla growth guidance provided similar correction of spinal deformity, equal number of complications, but with significantly less surgical procedures in patients treated for EOS when compared with MCGR instrumentation. MCGR provided with slightly better spinal growth during the distraction period. There were no significant differences between the quality of life assessments. 3.

Characterizing Use of Growth-friendly Implants for Early-onset Scoliosis: A 10-Year Update.

Growth-friendly treatment of early-onset scoliosis (EOS) has changed with the development and evolution of multiple devices. This study was designed to characterize changes in the use of growth-friendly implants for EOS from 2007 to 2017. We queried the Pediatric Spine Study Group database for patients who underwent index surgery with growth-friendly implants from July 2007 to June 2017. In 1298 patients, we assessed causes of EOS; preoperative curve magnitude; age at first surgery; patient sex; construct type; lengthening interval; incidence of "final" fusion for definitive treatment; and age at definitive treatment. α=0.05. From 2007 to 2017, the annual proportion of patients with idiopathic EOS increased from 12% to 33% (R=0.58, P=0.006). Neuromuscular EOS was the most common type at all time points (range, 33% to 44%). By year, mean preoperative curve magnitude ranged from 67 to 77 degrees, with no significant temporal changes. Mean (±SD) age at first surgery increased from 6.1±2.9 years in 2007 to 7.8±2.5 years in 2017 (R=0.78, P<0.001). As a proportion of new implants, magnetically controlled growing rods increased from <5% during the first 2 years to 83% in the last 2 years of the study. Vertically expandable prosthetic titanium ribs decreased from a peak of 48% to 6%; growth-guidance devices decreased from 10% to 3%. No change was seen in mean surgical lengthening intervals (range, 6 to 9 mo) for the 614 patients with recorded lengthenings. Final fusion was performed in 88% of patients who had undergone definitive treatment, occurring at a mean age of 13.4±2.4 years. From 2007 to 2017, neuromuscular EOS was the most common diagnosis for patients treated with growth-friendly implants. Patient age at first surgery and the use of magnetically controlled growing rods increased during this time. Preoperative curve magnitude, traditional growing rod lengthening intervals, and rates of final fusion did not change. Level II.

Proximal junction kyphosis after posterior spinal fusion for early-onset scoliosis.

Retrospective study. The aim of this study was to assess the presence of proximal junctional kyphosis (PJK) in our population of children with early-onset scoliosis (EOS) and to identify the predisposing factors for the development of PJK in the postoperative period after posterior spinal fusion (PSF). Few studies have been conducted to evaluate the incidence of proximal junction kyphosis (PJK) in children after early-onset scoliosis (EOS) after posterior spinal fusion (PSF). Overall, 114 pediatric patients aged < 10years who underwent surgery for scoliosis or kyphoscoliosis at a single center between 2013 and 2015 were evaluated. Forty-five patients submitted to PSF of five or more levels met the inclusion criteria. The sample included 12 female and 10 male patients. Mean age at surgery was 7years and 8months. PJK was observed in 22 patients (48.9%). Overall, the mean proximal junctional angle at 12 and 36months was 17.1° and 22°, respectively. The uppermost instrumented vertebra (UIV) with the highest PJK rate was T6-T7. The lowest instrumented vertebra (LIV) with the highest PJK rate was L2. Etiology was idiopathic in 4, neuromuscular in 11, congenital in 14, and syndromic in 16. According to underlying disorder, prevalence of PJK was 78% in those with a congenital, 50% in those with a syndromic, 12% in those with idiopathic, and 9% in those with a neuromuscular EOS. Surgical revision rate was 4% (one patient). Mean postoperative follow-up was of 3years and 4months (range 3-4years and 1month). Congenital and syndromic etiology, but not age at PJK onset or sex of the patient, significantly affected the incidence rate of PJK. The UIV with the highest PJK rate was T6-T7 and the LIV with the highest PJK rate was L2. The patients had a low surgical revision rate. Level IV.

Incidence of complications in the management of non-ambulatory neuromuscular early-onset scoliosis with a rib-based growing system: high- versus low-tone patients.

The purpose of this study is to evaluate whether patients with high-tone neuromuscular early-onset scoliosis have different surgical outcome and complication rate, when compared to patients with low-tone neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. This is a retrospective cohort study of 67 neuromuscular early-onset scoliosis patients, collected from a multicenter database, treated with a rib-to-pelvis rib-based dual growing system. All patients were divided into two groups: high tone and low tone. Pre-, intra- and postoperative data were compared between both groups. Complications were reported by a standardized system. Twenty-six high-tone and 41 low-tone patients were found homogeneous regarding gender, age at surgery, weight, height, estimated blood loss and surgery time. High-tone group (19/26 = 73.1%) experiences more postoperative complications than low-tone group (22/41 = 53.7%). Most common complications were infection, device migration, death and hardware failure. Permanent abandonment of rib-based growing technique and device removal was required in 21% of high-tone patients (P < 0.001). None of the low-tone patients required abandonment. High-tone patients had more complications than those with low tone in management of neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. A different surgical approach may be required to treat the high-tone neuromuscular early-onset scoliosis.

Achievement of Guided Growth in Children With Low-Tone Neuromuscular Early-Onset Scoliosis Using a Segmental Sublaminar Instrumentation Technique

Objectives/Summary of Background DataSegmental sublaminar spinal instrumentation without fusion results in guided growth and correction of deformity in early-onset scoliosis (EOS). The purpose of this study is to report the outcomes of a series of children with low-tone EOS that were treated surgically with a modification of a Luque trolley technique. MethodsThis study is a retrospective chart and radiographic review of a single-center series of 13 consecutive children who met inclusion criteria with documented progression of scoliosis greater than 25°. All children received surgical treatment with guided growth without fusion using a modified Luque trolley technique. The children's preoperative, postoperative, and most recent radiographs were assessed for Cobb angle, T1–T12 and T1–S1 height, and sagittal alignment including proximal junctional kyphosis. Surgimap spine software was used for calibration and measurement purposes. Complications and need for repeat and/or secondary surgical procedures were recorded. ResultsThe mean age at surgery was 7.4 years (4.6–10.5). On average, 15 segments (13–16) were instrumented. None of the children went on to a spontaneous fusion, and the average growth rate per year from T1–T12 and T1–S1 was 0.9 cm/y and 1.5 cm/y, respectively. The mean total growth from T1–T12 and T1–S1 was 22.3 cm (16.6–30.2) and 37.5 cm (30.1–46.4). A total of three additional surgeries were needed in two children to address complications. There were no mortalities. ConclusionsSublaminar guided growth is a safe and effective treatment in the Low Tone Neuromuscular subset of EOS. Follow-up studies failed to show signs of auto fusion, and implant failure was not observed in our cohort. All children displayed growth postoperatively without the need for multiple distraction-based surgeries. Guided growth minimizes the risks associated with multiple surgical procedures while maintaining correction and allowing for near-normal rates of spinal growth.

0016 - A Single Centre Experience With Magnetically Controlled Rods in Neuromuscular Early Onset Scoliosis: The Alderhey Experience

0016 - A Single Centre Experience With Magnetically Controlled Rods in Neuromuscular Early Onset Scoliosis: The Alderhey Experience

Spasticity is a Risk factor of Complications and Surgical Outcome in the Management of Early-Onset Neuromuscular Scoliosis with a Rib-Based Growing System

Spasticity is a risk factor for increased complications and decreased coronal plane correction in the management of neuromuscular Early-Onset Scoliosis with a RBGS.

Evaluating the Extent of Clinical Uncertainty Among Treatment Options for Patients with Early-Onset Scoliosis

Literature guiding the management of early-onset scoliosis consists primarily of studies with a low level of evidence. Evaluation of clinical equipoise (i.e., when there is no known superiority among treatment modalities) allows for prioritization of research efforts. The objective of this study was to evaluate areas of clinical uncertainty among pediatric spine surgeons regarding the treatment of early-onset scoliosis. Fourteen experienced pediatric spine surgeons participated in semistructured interviews to identify clinical variables that influence decision making in the treatment of early-onset scoliosis. A series of case scenarios of 315 patients with idiopathic and neuromuscular early-onset scoliosis was then developed to be representative of those encountered in clinical practice. Using an online survey, eleven surgeons selected their choice of eight treatment options for each case scenario. Associations between case characteristics and treatment choices were assessed with chi-square and logistic regression analysis. Participants then reviewed the areas of treatment uncertainty identified in the survey, nominated additional research questions of interest, and ranked their interest to further explore the identified research questions. Collective equipoise was identified in numerous scenarios in the survey spanning a range of ages and magnitudes of scoliosis, and additional questions were identified during the nominal group technique. Areas that had the greatest clinical uncertainty included the management of patients who have finished treatment with a growing-rod, timing of rod-lengthening intervals, and indications for spine-based and rib-based proximal instrumentation anchors. The use of rib anchors compared with spine-based anchors was ranked highly for consideration in future clinical trials. Variability in decision making with regard to the optimum treatment of certain subsets of patients with early-onset scoliosis reflects gaps in the available evidence. Structured consensus methods identified priorities for higher levels of research in this area of scoliosis. Higher-level studies, including randomized trials, should focus on answering the questions highlighted in this report.

Optimizing Safety and Outcomes in Spinal Deformity Surgery: Early-Onset Scoliosis

Several interventions can improve the natural history of progressive Early-Onset Scoliosis (EOS); however, each has its own specific indications and limitations. The treating surgeon should be aware of the risks and benefits of each intervention. The objective of the study was to review the current management of EOS and how to improve the safety and outcomes of different treatment methods. Pulmonary function correlates with the height of the thoracic spine (T1–T12). The growth of the thoracic spine to 18 Cm at the age of 5 and 22 Cm at the age of 10 will minimize the risk of pulmonary complications. Growing Rods (GR) technique has a wide application in EOS with different etiologies. Index GR surgery at an older age and using submuscular, dual growing rod, have reduced the rate of complications. Patients with abnormal thoracic kyphosis, and also with syndromic and neuromuscular EOS are at higher risk for development of complications. Aside from its original indication, thoracic insufficiency syndrome (TIS), Vertical Expandable Prosthetic Titanium Rib (VEPTR) can address different types of spinal deformity. Morbidity of the rib cradles is the main reason for revisions. Meticulous soft tissue coverage and surgical technique are of utmost importance for an effective and safe management. The goal of growth-guided techniques (eg, Shilla), a single time procedure, is to allow continuing growth with fewer returns to surgery. In selected cases, it can serve as an interim procedure before definitive treatment. Treatment of EOS is a long-term commitment for both the patient and treating surgeon. Careful and individualized treatment planning maximizes the safety and efficacy of the treatment for EOS. Knowledge of indications, capabilities, and drawbacks of each technique is the key to optimize the outcomes. Extensive research is underway to introduce less-invasive techniques to enhance the safety and reduce complications.