Growth-friendly Instrumentation Research Articles

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

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

Serum Titanium Levels Remain Elevated But Urine Titanium is Undetectable in Children With Early Onset Scoliosis Undergoing Growth-Friendly Surgical Treatment: A Prospective Study

Background: Elevated serum titanium levels have been found in patients with early onset scoliosis (EOS) treated with traditional growing rods (TGR), magnetically controlled growing rods (MCGR), and vertical expandable prosthetic titanium rib (VEPTR). No studies have investigated whether serum titanium remains persistently elevated and if titanium is excreted. Our purpose was to compare serum titanium levels in patients with EOS with growth-friendly instrumentation to age-matched controls and evaluate urine titanium and serial serum titanium levels in patients with EOS. Methods: This was a prospective case-control study. Patients with EOS with TGR, MCGR, or VEPTR underwent urine titanium and serial serum titanium collection at a minimum 6-month interval. Control patients did not have a history of metal implant insertion and underwent serum titanium collection before fracture fixation. Results: Twenty patients with EOS (6 TGR, 8 MCGR, and 6 VEPTR) and 12 controls were analyzed. The control group had no detectable serum titanium (0 ng/mL), whereas the patients with EOS had a median serum titanium of 4.0 ng/mL (P < 0.001). Analysis of variance showed significantly higher median serum titanium levels in the MCGR and VEPTR groups than the TGR group at time point 1 (5.5 vs 6.0 vs 2.0 ng/mL, P = 0.01) and time point 2 (6.5 vs 7.5 vs 2.0 ng/mL, P < 0.001). Binary comparisons showed a significant difference in serum titanium level between TGR and MCGR (time point 1: P = 0.026, time point 2: P = 0.011) and TGR and VEPTR (time point 1: P = 0.035, time point 2: P = 0.003). However, there was no difference between MCGR and VEPTR (time point 1: P = 0.399, time point 2: P = 0.492) even though the VEPTR group had a longer duration of follow-up (P = 0.001) and a greater number of lengthenings per patient at the first serum collection (P = 0.016). No patients with EOS had detectable urine titanium. Conclusions: Patients with EOS treated with titanium alloy growth-friendly instrumentation had elevated serum titanium levels compared with age-matched controls that persisted over time with no evidence of renal excretion. Additional studies are necessary to assess for local and systemic accumulation of titanium and the significance of long-term exposure to titanium in growing children. Level of Evidence: Level III, therapeutic.

Novel dual: rod plate system for EOS improves vertebral wedging and permits spinal growth

BackgroundTo determine whether single-stage, growth-friendly instrumentation with a plate-rod spinal system (PRSS) can substantially correct the deformity of EOS at surgery and continue to rectify the deformity throughout the growth period.MethodsAn observational study of 35 children with EOS treated by PRSS between February 2000 and October 2010 during a mean follow-up of 72 months. The mean age at surgery was 7 years. X-rays were taken preoperatively and postoperatively and at each follow-up. The Cobb angle, the apical vertebral wedge angle (AVWA), remaining rod lengths, maximal thoracic kyphosis and total T1-S1 heights were measured and compared.ResultsThirty-one patients, 9 boys and 22 girls, with a mean age of 7 years were completed follow-up. The Cobb angle changed from 64° to 36° after initial surgery and 26° at the last follow-up. The mean AVWA was 15° postoperatively and 5° at the last follow-up. The mean rod tail reserve length decreased from 53 mm immediately after surgery to 12 mm at the last follow-up. The mean preoperative maximum thoracic kyphosis was 41° and changed to 35° and 30° postoperatively and at latest follow-up, respectively. The mean preoperative T1–S1 height for all 32 patients was 52 mm acute lengthening and 122 mm of lengthening by the end of follow-up, respectively.ConclusionThe PRSS provided immediate correction of most of the deformity at surgery and continued to rectify remaining scoliosis during the growth period. AVWA may be a useful method for monitoring the function of the PRSS in EOS.

Update on the Diagnosis and Management of Early-onset Scoliosis.

In this article, we review the most recent advancements in the approaches to EOS diagnosis and assessment, surgical indications and options, and basic science innovation in the space of early-onset scoliosis research. Early-onset scoliosis (EOS) covers a diverse, heterogeneous range of spinal and chest wall deformities that affect children under 10 years old. Recent efforts have sought to examine the validity and reliability of a recently developed classification system to better standardize the presentation of EOS. There has also been focused attention on developing safer, informative, and readily available imaging and clinical assessment tools, from reduced micro-dose radiographs, quantitative dynamic MRIs, and pulmonary function tests. Basic science innovation in EOS has centered on developing large animal models capable of replicating scoliotic deformity to better evaluate corrective technologies. And given the increased variety in approaches to managing EOS in recent years, there exist few clear guidelines around surgical indications across EOS etiologies. Despite this, over the past two decades, there has been a considerable shift in the spinal implant landscape toward growth-friendly instrumentation, particularly the utilization of MCGR implants. With the advent of new biological and basic science treatments and therapies extending survivorship for disease etiologies associated with EOS, the treatment for EOS has steadily evolved in recent years. With this has come a rising volume and variation in management options for EOS, as well as the need for multidisciplinary and creative approaches to treating patients with these complex and heterogeneous disorders.

Effects of distraction-based distal fixation on pelvic parameter development in early onset scoliosis.

Prior studies have suggested that distraction-based treatment for early onset scoliosis (EOS) may impede the natural development of the sagittal spinal alignment and pelvic parameters. However, to date no study has investigated the effect of distal fixation on pelvic development. Ambulatory children with EOS undergoing index distraction-based treatment with distal fixation below T11 were retrospectively reviewed. Patients with distal fixation to the pelvis were identified and compared to children with Spine-based fixation at T12-L5. Radiographic measurements were performed for coronal and sagittal alignment in addition to pelvic parameters (pelvic incidence (PI), pelvic tilt (PT), and sacral slope (SS) and compared at initial presentation, first erect radiograph, and at 2years following instrumentation. 33 ambulatory children were identified with a minimum of 2-year follow-up (25 female, average 6.59 ± 2.6years), with 33% (N = 11) instrumented to the pelvis (54.4% female, average 4.42 ± 2.2years, initial Cobb 76.1°). Children in the pelvis cohort were significantly younger at treatment initiation (P < 0.001). There was no significant difference in PI at the study time periods, however, there was a significant change in PI between presentation and 2-year follow-up with the pelvic fixation demonstrating a mean 12.3° decrease in PI vs a 3.8° increase in the spine-based cohort (P = 0.027). Distal fixation to the pelvis in ambulatory children with EOS treated with growth-friendly instrumentation was associated with a mean decrease in PI of 12.3° that developed over the 2-year treatment duration. Further research is needed to investigate the long-term implications of these findings on pelvic and spinal development.

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.

Pelvic fixation is not always necessary in children with cerebral palsy scoliosis treated with growth-friendly instrumentation.

A previous study showed that patients with neuromuscular scoliosis who underwent fusion to L5 had excellent coronal curve correction and improvement in pelvic obliquity (PO) when preoperative L5 tilt was < 15°. Our purpose was to identify indications to exclude the pelvis in children with cerebral palsy (CP) scoliosis treated with growing-friendly instrumentation. In a retrospective cohort study, children with CP scoliosis treated with TGR, MCGR, or VEPTR with minimum 2-year follow-up were identified from a multicenter database. 27 patients with distal spine anchors (DSA) and 71 patients with distal pelvic anchors (DPA) placed at the index surgery were analyzed. The DSA group had a lower pre-index PO (9° vs 16°, P = 0.0001). Most recent radiographic data were similar except the DSA patients had a smaller major curve (47° vs 58°, P = 0.038). 6 (22%) DSA patients underwent extension of the instrumentation to the pelvis (DSA-EXT), most commonly at final fusion (5 patients). DSA-EXT patients had a higher pre-index L5 tilt than patients who did not require extension (DSA-NO EXT) (19° vs 10°, P = 0.009). Sub-analysis showed a lower major curve at most recent follow-up in the DSA-EXT group compared to the DPA group (33° vs 58°, P = 0.021). The DSA-EXT group had a higher number of complications per patient compared to the DSA-NO EXT group (2.3 vs 1.1, P = 0.029). Pre-index L5 tilt ≤ 10° and PO < 10° may be indications to exclude the pelvis in children with CP scoliosis treated with growth-friendly instrumentation. DSA may provide better long-term control of the major curve than DPA.

Early outcomes of growth friendly instrumentation in children with Williams syndrome.

Although scoliosis and kyphosis have been associated with Williams Syndrome (WS), no previous literature has reported on surgical treatment for early onset scoliosis (EOS) in WS. The aim of this case series is to report on the outcomes of spine deformity surgery in patients with EOS and WS and any perioperative anesthetic or cardiovascular complications. One multicenter database was queried for all patients with WS who underwent growth-friendly (GF) treatment before age 12 between 2000 and 2017. Demographics, surgical, and growth-friendly data were queried. Radiographs were measured for curve magnitude, T1-T12 length, and T1-S1 length. Seven patients were analyzed (3 males, 4 females). Patients were at a median age of 2.8years at initial surgery with median follow-up 3.6years (range 2.0-12years) after index surgery. The initial surgical treatments were as follows: 2 traditional growing rods (TGR), 2 magnetically controlled growing rods (MCGR), and 3 vertical expandable prosthetic titanium ribs (VEPTR). The median duration of growth-friendly treatment was 5.0years (range, 2.6-10.4years) with a median number of 9 device lengthenings. The median improvement in coronal curve magnitude from preoperative to most recent follow-up was 19° (range, 54°-9°). Three patients have completed GF treatment: one underwent definitive fusion, and two are under observation with apparent spontaneous fusion and retain the original GF implants. No peri-operative anesthetic or cardiovascular complications occurred. Few studies have reported on surgical outcomes in WS patients with EOS. In this case series, 6/7 patients experienced curve improvement with growth-friendly spine instrumentation. This study suggests that growth-friendly instrumentation for severe EOS in WS can be used for control of spinal deformity while allowing for further growth. Associated complications were typical of distraction-based EOS surgical treatment. There were 62 total procedures with general anesthesia, but no perioperative cardiac complications occurred.

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.

The Effect of Surgeon Experience on Outcomes Following Growth Friendly Instrumentation for Early Onset Scoliosis.

The purpose of this study was to utilize a multicenter, multisurgeon cohort to assess the effect of surgeon experience on outcomes of growth friendly instrumentation (GFI) in early onset scoliosis (EOS). We hypothesized that unplanned return to the operating room (UPROR), estimated blood loss (EBL), and surgical time would be greater amongst early career surgeons (ECSs) when compared with advanced career surgeons (ACSs). An international pediatric spine database was queried for patients ages 2 to 10 years treated by posterior distraction-based GFI with at least of 2-year follow up. Two groups were created for analysis based on surgeon experience: ECSs (with ≤10 y of experience) and ACSs (with >10 y of experience). The primary outcome was UPROR. Additional outcomes included: operating room time, EBL, neurological deficits, infection rate, hardware failure, and the Early Onset Scoliosis Questionnaire (EOSQ-24). Subgroup analysis was performed for further assessment based on procedure type, superior anchor type, etiology, and curve severity. A total of 960 patients met inclusion criteria including 243 (25.3%) treated by ECS. Etiology, sex, superior anchor, and EOSQ-24 scores were similar between groups (P>0.05). There were no clinically significant differences in patient age or preoperative major coronal curve. UPROR (35.8% vs. 32.7%, P=0.532), infection (17.0% vs. 15.6%, P=0.698), operating room time (235 vs. 231 min, P=0.755), and EBL (151 vs. 155 mL, P=0.833) were comparable between ECS and ACS groups. The frequency of having at least 1 complication was relatively high but comparable among groups (60.7% vs. 62.6%, P=0.709). EOSQ-24 subdomain scores were similar between groups at 2-year follow-up (P>0.05). Subgroup analysis revealed that ECS had increased surgical time compared with ACS in severe curves >90 degrees (270 vs. 229 min, P=0.05). This study represents the first multicenter assessment of surgeon experience on outcomes in EOS. Overall, surgeon experience did not significantly influence UPROR, complication rates, EBL, or surgical time associated with GFI in this cohort of EOS patients. Level III.

The Impact of Unplanned Return to the Operating Room on Health-related Quality of Life at the End of Growth-friendly Surgical Treatment for Early-onset Scoliosis.

Limiting complications, especially unplanned return to the operating room (UPROR), is a major focus in the surgical management of early-onset scoliosis (EOS). Although UPROR remains common in this population, its effect on long-term health-related quality of life (HRQoL) remains unclear. The purpose of this study was to investigate the association between UPROR and end-of-treatment HRQoL in EOS patients treated with growth-friendly instrumentation. Patients with EOS who underwent growth-friendly instrumentation at age less than 10 years from 1993 to 2018, and completed treatment, were identified in a multicenter EOS registry. UPROR events were recorded, and end-of-treatment (defined as skeletal maturity and/or definitive spinal fusion) HRQoL was assessed via the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24). A total of 825 patients were identified, and 325 patients (age at surgery: 6.4 y, follow-up: 8.1 y) had end-of-treatment HRQoL data necessary for our investigation. Overall, 129/325 (39.7%) patients experienced 264 UPROR events; the majority (54.2%) were implant-related. Aside from age and etiology, no other variables were determined to be confounders or effect modifiers. Congenital patients with UPROR had worse pain/discomfort by 10.4 points (P=0.057) and worse pulmonary function by 7.8 points (P=0.102) compared with non-UPROR patients adjusting for age. Neuromuscular patients with UPROR had worse pulmonary function by 10.1 points compared with non-UPROR patients adjusting for age (P=0.037). Idiopathic and syndromic patients with UPROR reported consistently worse domain scores than their non-UPROR counterparts, but smaller (<5-point) differences were seen. UPROR during growth-friendly surgical treatment for EOS is associated with worse HRQoL in all patients, but particularly in those with neuromuscular or congenital etiologies. Ongoing efforts to avoid UPROR are critical. Level II. This is a multicenter retrospective cohort study investigating the effect of UPROR on HRQoL (prognostic study).

Early Complications in Vertical Expandable Prosthetic Titanium Rib and Magnetically Controlled Growing Rods to Manage Early Onset Scoliosis.

The aim of this study is to examine whether surgical treatment of early onset scoliosis (EOS) with magnetically controlled growing rods (MCGRs) or a vertical expandable prosthetic titanium rib (VEPTR) resulted in fewer short-term (24 months) complications and reoperations. EOS is a challenging problem for spine surgeons that has been managed with different growth-friendly instrumentation systems. Although rib-based devices encourage spinal growth via regular lengthening, the high rate of complications and reoperations leads us to use spine-based devices such as MCGRs to mitigate this concern. A total of 35 EOS patients were included in the study. Twenty patients were included in the VEPTR group, and 15 patients were included in the MCGR group. Demographic data and 2 years of postoperative complications and reoperations were reviewed retrospectively. As secondary outcomes, radiographic outcomes were reported preoperatively and 1 year after surgery. Indications for this technique and complications were collected from the charts. Demographic data showed no significant differences between the 2 groups. Significant differences were found in the complications rate at 2 years, with 65% complications in the VEPTR group and 13.3% complications in the MCGR group (P < .001). The reoperation rate at 2 years was also significantly higher in the VEPTR group, with 50% versus 13.3% in the MCGR group (P = .0009). As secondary outcomes, radiological parameters such as main curve Cobb angle correction (P = .001) and apical vertebral translation (P = .002) were significantly higher in the MCGR group. Significant differences were also found in sagittal profile parameters; T1-T12 and T1-S1 were significantly higher in the MCGR group (P < .001). According to our results, VEPTR has significantly higher complication and reoperation rates at 2 years postsurgery compared with MCGR. 4.

Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita.

Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes. Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression. Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS. Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

The potential of spring distraction to dynamically correct complex spinal deformities in the growing child

PurposeCurrent treatment of progressive early onset scoliosis involves growth-friendly instrumentation if conservative treatment fails. These implants guide growth by passive sliding or repeated lengthenings. None of these techniques provide dynamic correction after implantation. We developed the spring distraction system (SDS), by using one or multiple compressed springs positioned around a standard sliding rod, to provide active continuous distraction of the spine to stimulate growth and further correction. The purpose of this study was to determine feasibility and proof of concept of the SDS.MethodsWe developed a versatile, dynamic spring distraction system for patients who would benefit from active continuous distraction. This prospective case series evaluates four patients with exceptional and progressive congenital spine deformities.ResultsFour patients had a mean age of 6.8 years at surgery with a mean follow-up of 36 months (range 25–45). The mean progressive thoracic lordosis, which was the reason for initiating surgical treatment in two patients, changed from 32° lordosis preoperatively to 1° kyphosis post-operatively. During follow-up, this further improved to 32° thoracic kyphosis. In the two other patients, with cervicothorcacic scoliosis, the main coronal curve improved from 79° pre-operatively to 56° post-operatively and further improved to 42°. The mean T1-S1 spine growth during follow-up for all patients was 1.3 cm/year. There was one reoperation because of skin problems and no device-failures.ConclusionThese early results show the feasibility and the proof of concept of spring-based distraction as a dynamic growth-enhancing system with the potential of further correction of the deformity after implantation.

Improvement of Pulmonary Function Measured by Patient-reported Outcomes in Patients With Spinal Muscular Atrophy After Growth-friendly Instrumentation.

Children with spinal muscular atrophy (SMA) sustain a progressive reduction in pulmonary function (PF) related to both muscular weakness and the concomitant effects of spinal deformity on the thorax. Growth-friendly instrumentation is commonly utilized for younger patients with scoliosis and SMA to halt the progression of spinal curvature, but its effect on PF in these patients has not previously been investigated. Using the change in Early Onset Scoliosis 24-Item Questionnaire (EOSQ-24) PF subdomain scores, the authors will investigate whether PF improves in patients with SMA after a growth-friendly intervention. This was a multicenter retrospective cohort study from 2 international registries of patients with SMA undergoing spinal deformity surgery from 2005 to 2015. Data collected were age, sex, degree of major coronal curve, type of growth-friendly construct, forced vital capacity (FVC), and EOSQ-24 scores at the patient's preoperative, 1-year postoperative, and 2-year postoperative visits. Differences in EOSQ-24 PF scores and FVC between baseline and postoperative assessment were examined by paired tests. A total of 74 patients were identified (mean age, 7.6±2.3 y, major curve 68.1±22.4 degrees, 51.4% female individuals). The mean EOSQ-24 PF scores improved significantly from 70.6 preoperatively to 83.6 at 1 year (P=0.092) and 86.5 at 2 years postoperatively (P=0.020). The scores in patients with rib-based constructs showed steeper increases at 1-year assessments than those in patients with spine-based constructs. The mean paired FVC value decreased from 63.9% predicted preoperatively, to 57.6% predicted at 1 year postoperatively (P=0.035), and 61.9% predicted preoperatively, to 56.3% predicted at 2 years postoperatively (P=0.178). Patients with SMA who received growth-friendly instrumentation did experience improvements in PF as measured by EOSQ-24 assessing the caregivers' perception. Given the uncertain reliability of PFTs in this young population, EOSQ-24 is an important tool for measuring improvements in health-related quality of life. Level III-retrospective study.

The Association Between the Utilization of Traction and Postoperative Complications Following Growing Rod Instrumentation for Early-onset Scoliosis.

Preoperative and/or intraoperative traction have been proposed as adjunctive methods to limit complications associated with growth-friendly instrumentation for early-onset scoliosis (EOS). By gradually correcting the deformity before instrumentation, traction can, theoretically, allow for better overall correction without the complications associated with the immediate intraoperative correction. The purpose of this multicenter study was to investigate the association between preoperative/intraoperative traction and complications following growth-friendly instrumentation for EOS. Patients with EOS who underwent growth rod instrumentation before 2017 were identified from 2 registries. Patients were divided into 2 groups: preoperative traction group versus no preoperative traction group. A subgroup analysis was done to compare intraoperative traction only versus no traction. Data was collected on any postoperative complication from implantation to up to 2 years postimplantation. Of 381 patients identified, 57 (15%) and 69 (18%) patients received preoperative and intraoperative traction, respectively. After adjusting for etiology and degree of kyphosis, there was no evidence to suggest that preoperative halo traction reduced the risk of any complication following surgical intervention. Although not statistically significant, a subgroup analysis of patients with severe curves demonstrated a trend toward a markedly reduced hardware failure rate in patients undergoing preoperative halo traction [preoperative traction: 1 (3.1%) vs. no preoperative traction: 11 (14.7%), P=0.083]. Nonidiopathic, hyperkyphotic patients treated with intraoperative traction were 61% less likely to experience any postoperative complication (P=0.067) and were 74% (P=0.091) less likely to experience an unplanned return to the operating room when compared with patients treated without traction. This multicenter study with a large sample size provides the best evidence to date of the association between the use of traction and postoperative complications. Our results justify the need for future Level I studies aimed at characterizing the complete benefit and risk profile for the use of traction in surgical intervention for EOS. Level III.

Spinal Deformity in Sotos Syndrome: First Results of Growth-friendly Spine Surgery.

Sotos syndrome (SS), or cerebral gigantism, describes children with macrocephaly, craniofacial abnormalities, general overgrowth, ligamentous laxity, developmental delay, and neurological disabilities. Fewer than 500 cases have been reported since Sotos and colleagues described the condition in 1964 and no literature exists on the management of spinal deformity in children under 10 years old.The aims of this study were: (1) to characterize the presentation of spinal deformities in patients with SS; and (2) to provide preliminary results of growth-friendly instrumentation (GFI) in these children. Thirteen children (9 boys) with SS and minimum of 2-year follow-up were identified from 2 multicenter early-onset scoliosis (EOS) databases (1997-2017). Mean age at index surgery and follow-up duration were 5.0 years (range, 1.8 to 10 y) and 7.2 years (range, 2.1 to 14.9 y), respectively. Patients underwent GFI for a mean of 5.7 years (range, 2 to 10.2 y), with an average of 9 lengthenings (range, 2 to 18). Definitive spinal fusion was performed in 4 patients (31%). Major curve magnitude, T1-T12 and T1-S1 lengths, thoracic kyphosis, and lumbar lordosis were evaluated preindex, postindex, latest GFI, and postfusion, when possible. Five thoracolumbar (38%), 4 double major (31%), 2 main thoracic (15%), and 2 double thoracic curves (15%) were seen that spanned a mean of 6.8 levels (5 to 9). Major curves improved 36% (range, 5% to 71%), from a mean of 71 degrees (range, 48 to 90 degrees) to 46 degrees (range, 20 to 73 degrees) postindex surgery (P<0.001). Major curves remained stable at a mean of 52 degrees (range, 20 to 87 degrees) at latest GFI (P=0.36). True T1-T12 and T1-S1 growth velocities during GFI were 0.5 mm/mo (range, 0.4 to 0.8 mm/mo) and 0.8 mm/mo (range, 0.1 to 2.1 mm/mo), respectively. Twenty-six complications occurred in 9 patients (69%) averaging 2 complications per patient (range, 0 to 7). This is the first study to evaluate the outcomes of GFI in children with SS and EOS. Compared with published data for outcomes of GFI in EOS, children with SS may have less major curve correction. Growth-friendly surgery remains an effective treatment method for EOS in patients with SS. Level IV-retrospective case-series.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.

Elevated Serum Titanium Levels in Children With Early Onset Scoliosis Treated With Growth-friendly Instrumentation.

A previous study showed significantly higher serum titanium levels in patients with early-onset scoliosis (EOS) treated with traditional growing rods (TGR) and magnetically controlled growing rods (MCGR) compared with controls. Children with vertical expandable prosthetic titanium rib (VEPTR) were not assessed. The purpose of this study was to compare serum titanium levels in EOS patients treated with TGR, MCGR, and VEPTR. We hypothesized that EOS patients treated with all forms of growth-friendly instrumentation (GFI) have elevated serum titanium levels. This was a prospective cross-sectional case series. Serum titanium levels were collected from patients with GFI who were enrolled in an EOS database. Blood samples were collected at a clinic visit or lengthening/exchange procedure between April and December 2018. The normal range for serum titanium is 0 to 1 ng/mL. Analyses were conducted using analysis of variance and Bonferroni post hoc test. A total of 23 patients (2 TGR, 8 MCGR, 13 VEPTR) were analyzed. There was a significant difference in age at the time of blood sample collection (12.5 vs. 9.8 vs. 7.5 y, P=0.015) and serum titanium level (1.5 vs. 4.5 vs. 7.6 ng/mL, P=0.021) between TGR, MCGR, and VEPTR, respectively. All of the MCGR and VEPTR patients had a serum titanium level ≥2 ng/mL. Binary comparisons showed that VEPTR had a significantly higher serum titanium level than TGR (P=0.046). There was no difference in serum titanium level when MCGR was compared with TGR and VEPTR. Time from implant insertion to blood sample collection, number of rods currently implanted, total number of rods implanted throughout treatment, and number of lengthenings per patient was similar between the groups. Elevated serum titanium levels may be present in EOS patients treated with all forms of GFI. Although our TGR patients had indwelling implants for the longest period of time, they had the lowest serum titanium level. Repetitive chest wall motion during respiration may lead to continued wear and metal ion release with VEPTR. Level II-therapeutic.

Growth-friendly Instrumentation for the Treatment of Early-onset Scoliosis in Marfan Syndrome

Growth-friendly Instrumentation for the Treatment of Early-onset Scoliosis in Marfan Syndrome