The Fate of The Broken Tether: How Do Curves Treated with Vertebral Body Tethering Behave After Tether Breakage?

STUDY DESIGN: Retrospective, Multicenter. OBJECTIVE: Assess curve progression and occurrence of revision surgery following tether breakage after vertebral body tethering (VBT). SUMMARY OF BACKGROUND DATA: Tether breakage after VBT is common with rates up to 50% reported. In these cases, it remains unknown whether the curve will progress or remain stable. METHODS: Adolescent and juvenile idiopathic scoliosis patients in a multicenter registry with ≥2 year-follow-up after VBT were reviewed. Broken tethers were listed as postoperative complications and identified by increased screw divergence of >5° on serial radiographs. Revision procedures and curve magnitude at subsequent visits were recorded. RESULTS: Of 186 patients who qualified for inclusion, 84 (45.2%) patients with tether breakage were identified with a mean age at VBT of 12.4±1.4 years and mean curve magnitude at index procedure of 51.8°±8.1°. Tether breakage occurred at a mean of 30.3±11.8 months and mean curve of 33.9°±13.2°. Twelve patients (12/84, 14.5%) underwent 13 revision procedures after VBT breakage, including 6 tether revisions and 7 conversions to fusion. All tether revisions occurred within 5 months of breakage identification. No patients with curves <35° after breakage underwent revision. Revision rate was greatest in skeletally immature (Risser 0-3) patients with curves ≥35° at time of breakage (Risser 0-3: 9/17, 53% vs. Risser 4-5: 3/23, 13%, P=0.01).Curves increased by 3.1° and 3.7° in the first and second year, respectively. By two years, 15/30 (50%) progressed >5° and 8/30 (26.7%) progressed greater than 10°. Overall, 66.7% (40/60) reached a curve magnitude >35° at their latest follow-up, and 14/60 (23.3%) reached a curve magnitude greater than 45°. Skeletal maturity did not affect curve progression after tether breakage (P>0.26), but time to rupture did (P=0.048). CONCLUSION: While skeletal immaturity and curve magnitude were not independently associated with curve progression, skeletally immature patients with curves ≥35° at time of rupture are most likely to undergo additional surgery. Most patients can expect progression at least 5° in the first two years after tether breakage, though longer-term behavior remains unknown. LEVEL OF EVIDENCE: III.

How will early onset scoliosis surgery affect my child's future as a young adult? A follow-up study using patient-reported outcome measures.

PURPOSE: Using patient-reported outcome measures (PROMs), this study was undertaken to determine how well patients with early onset scoliosis (EOS) fare in adulthood. METHODS: Among eight healthcare centers, 272 patients (≥ 18 years) surgically managed for EOS (≥ 5 years) completed the Scoliosis Research Society (SRS)-22r, Functional Assessment of Chronic Illness Therapy-10 (FACIT-Dyspnea-10), and Short Form (SF)-12. Functional and demographic data were collected. RESULTS: The response rate was 40% (108/272). EOS etiologies were congenital (45%), neuromuscular (20%), idiopathic (20%) syndromic (11%), and unknown (4%). All patients scored within normal limits on the FACIT-Dyspnea-10 pulmonary (no breathing aids, 78%; no oxygen, 92%). SF-12 physical health scores and most SRS-22r domains were significantly decreased (p < 0.05 and p < 0.001, respectively) compared with normative values. SF-12 and SRS-22r mental health scores (MHS) were lower than normative values (p < 0.05 and p < 0.02, respectively). Physical health PROMs varied between etiologies. Treatment varied by etiology. Patients with congenital EOS were half as likely to undergo definitive fusion. There was no difference between EOS etiologies in SF-12 MHS, with t scores being slightly lower than normative peers. CONCLUSION: Good long-term physical and social function and patient-reported quality of life were noted in surgically managed patients. Patients with idiopathic EOS physically outperformed those with other etiologies in objective and PROM categories but had similar MHS PROMs. Compared to normative values, EOS patients demonstrated decreased long-term physical capacity, slightly lower MHS, and preserved cardiopulmonary function. LEVEL OF EVIDENCE: Level IV Case Series.

Lowest instrumented vertebrae in early onset scoliosis: is there a role for a more selective approach?

PURPOSE: This purpose of this study was to assess the impact of patient and implant characteristics on LIV selection in ambulatory children with EOS and to assess the relationship between the touched vertebrae (TV), the last substantially touched vertebrae (LSTV), the stable vertebrae (SV), the sagittal stable vertebrae (SSV), and the LIV. METHODS: A multicenter pediatric spine database was queried for patients ages 2-10 years treated by growth friendly instrumentation with at least 2-year follow up. The relationship between the LIV and preoperative spinal height, curve magnitude, and implant type were assessed. The relationships between the TV, LSTV, SV, SSV, and the LIV were also evaluated. RESULTS: Overall, 281 patients met inclusion criteria. The LIV was at L3 or below in most patients with a lumbar LIV: L1 (9.2%), L2 (20.2%), L3 (40.9%), L4 (29.5%). Smaller T1 - T12 length was associated with more caudal LIV selection (p = 0.001). Larger curve magnitudes were similarly associated with more caudal LIV selection (p = < 0.0001). Implant type was not associated with LIV selection (p = 0.32) including MCGR actuator length (p = 0.829). The LIV was caudal to the TV in 78% of patients with a TV at L2 or above compared to only 17% of patients with a TV at L3 or below (p < 0.0001). CONCLUSIONS: Most EOS patients have an LIV of L3 or below and display TV-LIV and LSTV-LIV incongruence. These findings suggest that at the end of treatment, EOS patients rarely have the potential for selective thoracic fusion. Further work is necessary to assess the potential for a more selective approach to LIV selection in EOS. LEVEL OF EVIDENCE: III.

A Comparative Analysis of Revision Surgery Before or After 2 Years After Graduation From Growth-friendly Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth-friendly (GF) surgery for early onset scoliosis, patients are termed graduates: they undergo a spinal fusion, are observed after final lengthening with GF implant maintenance, or are observed after GF implant removal. The purpose of this study was to compare the rates of and reasons for revision surgery in two cohorts of GF graduates: before or after 2 years of follow-up from graduation. METHODS: A pediatric spine registry was queried for patients who underwent GF spine surgery with a minimum of 2 years of follow-ups after graduation by clinical and/or radiographic evidence. Scoliosis etiology, graduation strategy, number of, and reasons for revision surgery were queried. RESULTS: There were 834 patients with a minimum of 2-year follow-up after graduation who were analyzed. There were 241 (29%) congenital, 271 (33%) neuromuscular, 168 (20%) syndromic, and 154 (18%) idiopathic. 803 (96%) had traditional growing rod/vertical expandable titanium rib as their GF construct and 31 (4%) had magnetically controlled growing rod. Five hundred ninety-six patients (71%) underwent spinal fusion at graduation, 208 (25%) had GF implants retained, and 30 (4%) had GF implants removed.In the entire cohort, there were 108/834 (13%) patients who underwent revision surgery. Of the revisions, 71/108 (66%) occurred as acute revisions (ARs) between 0 and 2 years from graduation (mean 0.6 y), and the most common AR indication was infection (26/71, 37%). The remaining 37/108 (34%) patients underwent delayed revision (DR) surgery >2 years (mean 3.8 y) from graduation, and the most common DR indication was implant issues (17/37, 46%).Graduation strategy affected revision rates. Of the 596 patients with spinal fusion as a graduation strategy, 98/596 (16%) underwent revision, compared with only 8/208 (4%) patients who had their GF implants retained, and 2/30 (7%) that had their GF implants removed ( P ≤ 0.001).A significantly higher percentage of the ARs had a spinal fusion as the graduation strategy (68/71, 96%) compared with 30/37 DRs, (81%, P = 0.015). In addition, the 71 patients who underwent AR undergo more revision surgeries (mean: 2, range: 1 to 7) than 37 patients who underwent DR (mean: 1, range: 1 to 2) ( P = 0.001). CONCLUSION: In this largest reported series of GF graduates to date, the overall risk of revision was 13%. Patients who undergo a revision at any time, as well as ARs in particular, are more likely to have a spinal fusion as their graduation strategy. Patients who underwent AR, on average, undergo more revision surgeries than patients who underwent DR. LEVEL OF EVIDENCE: Level III, comparative.

Rib-to-spine and rib-to-pelvis magnetically controlled growing rods: does the law of diminishing returns still apply?

PURPOSE: The Law Of Diminishing Returns (LODR) has been demonstrated for traditional growing rods, but there is conflicting data regarding the lengthening behavior of Magnetically Controlled Growing Rods (MCGR). This study examines a cohort of patients with early-onset scoliosis (EOS) with rib-to-spine or rib-to-pelvis-based MCGR implants to determine if they demonstrate the LODR, and if there are differences in lengthening behaviors between the groups. METHODS: A prospectively collected multicenter EOS registry was queried for patients with MCGR with a minimum 2-year follow-up. Patients with rib-based proximal anchors and either spine- or pelvis-based distal anchors were included. Patients with non-MCGR, unilateral constructs, < 3 lengthenings, or missing > 25% datapoints were excluded. Patients were further divided into Primary-MCGR (pMCGR) and Secondary-MCGR (sMCGR). RESULTS: 43 rib-to-spine and 31 rib-to-pelvis MCGR patients were included. There was no difference in pre-implantation, post-implantation and pre-definitive procedure T1-T12 height, T1-S1 height, and major Cobb angles between the groups (p > 0.05). Sub-analysis was performed on 41 pMCGR and 19 sMCGR rib-to-spine patients, and 31 pMCGR and 17 sMCGR rib-to-pelvis patients. There is a decrease in rod lengthenings achieved at subsequent lengthenings for each group: rib-to-spine pMCGR (rho = 0.979, p < 0.001), rib-to-spine sMCGR (rho = 0.855, p = 0.002), rib-to-pelvis pMCGR (rho = 0.568, p = 0.027), and rib-to-pelvis sMCGR (rho = 0.817, p = 0.007). Rib-to-spine pMCGR had diminished lengthening over time for idiopathic, neuromuscular, and syndromic patients (p < 0.05), with no differences between the groups (p > 0.05). Rib-to-pelvis pMCGR neuromuscular patients had decreased lengthening over time (p = 0.01), but syndromic patients had preserved lengthening over time (p = 0.65). CONCLUSION: Rib-to-spine and rib-to-pelvis pMCGR and sMCGR demonstrate diminished ability to lengthen over subsequent lengthenings.

Preoperative left shoulder elevation > 1 cm is predictive of severe postoperative shoulder imbalance in early onset idiopathic scoliosis patients treated with growth-friendly instrumentation.

PURPOSE: Prior research has demonstrated the influence of preoperative shoulder elevation (SE), proximal thoracic curve magnitude, and upper instrumented vertebra (UIV) on shoulder balance after PSF for AIS. Our purpose was to evaluate the impact of these factors on shoulder balance in early onset idiopathic scoliosis (EOIS) patients treated with growth-friendly instrumentation. METHODS: This was a multicenter retrospective review. Children with EOIS treated with dual TGR, MCGR, or VEPTR and minimum 2-year follow-up were identified. Demographics and radiographic/surgical data were collected. RESULTS: 145 patients met inclusion criteria: 74 had right SE (RSE), 49 left SE (LSE), and 22 even shoulders (EVEN) preoperatively. Mean follow-up was 5.3 years (range, 2.0-13.1 years). The LSE group had a larger pre-index mean main thoracic curve (p = 0.021) but no difference was observed between groups at the post-index or most recent timepoints. RSE patients with UIV of T2 were more likely to have balanced shoulders post-index than patients with UIV of T3 or T4 (p = 0.011). Pre-index radiographic shoulder height (RSH) was predictive of post-index shoulder imbalance ≥ 2 cm in the LSE group (p = 0.007). A ROC curve showed a cut-off of 1.0 cm for RSH. 0/16 LSE patients with pre-index RSH < 1.0 cm had post-index shoulder imbalance ≥ 2 cm compared to 8/28 (29%) patients with pre-index RSH > 1.0 cm (p = 0.006). CONCLUSION: Preoperative LSE > 1.0 cm is predictive of shoulder imbalance ≥ 2 cm after insertion of TGR, MCGR, or VEPTR in children with EOIS. In patients with preoperative RSE, UIV of T2 resulted in a higher likelihood of balanced shoulders postoperatively.

Comparison of Distal Spine Anchors and Distal Pelvic Anchors in Children With Hypotonic Neuromuscular Scoliosis Treated With Growth-friendly Instrumentation.

BACKGROUND: Lower preoperative pelvic obliquity (PO) and L5 tilt have been associated with good radiographic outcomes when the fusion ended short of the pelvis in children with neuromuscular scoliosis (NMS). Our purpose was to identify indications to exclude the pelvis in children with hypotonic NMS treated with growth-friendly instrumentation. METHODS: This was a multicenter retrospective review. Children with spinal muscular atrophy and muscular dystrophy treated with dual traditional growing rod, magnetically controlled growing rod, or vertical expandable prosthetic titanium rib with minimum 2-year follow-up after the index surgery were identified. RESULTS: A total of 125 patients met the inclusion criteria. Thirty-eight patients had distal spine anchors (DSAs) and 87 patients had distal pelvic anchors (DPAs) placed at the index surgery. Demographics and length of follow-up were similar between the groups but there was a greater percentage of DPA patients who were nonambulatory [79 patients (91%) vs. 18 patients (47%), P <0.0001]. Preindex radiographic measures were similar except the DSA patients had a lower PO (11 vs. 19 degrees, P =0.0001) and L5 tilt (8 vs. 12 degrees, P =0.001). Postindex and most recent radiographic data were comparable between the groups. There was no difference in the complication and unplanned returns to the operating room rates.Subanalysis of the DSA group based on ambulatory status showed similar radiographic measures except the ambulatory patients had a lower PO at all time points (preindex: 5 vs. 16 degrees, P =0.011; postindex: 6 vs. 10 degrees, P =0.045; most recent follow-up: 5 vs. 14 degrees, P =0.028). Only 1 ambulatory DSA patient had a PO ≥10 degrees at most recent follow-up compared with 6 nonambulatory DSA patients. Three (8%) DSA patients, all nonambulatory, underwent extension of their instrumentation to the pelvis. CONCLUSIONS: Pelvic fixation should be strongly considered in nonambulatory children with hypotonic NMS treated with growth-friendly instrumentation. At intermediate-term follow-up, revision surgery to include the pelvis was rare but DSAs do not seem effective at maintaining control of PO in nonambulatory patients. DSA and DPA were equally effective at maintaining major curve control, and complication and unplanned returns to the operating room rates were similar. LEVEL OF EVIDENCE: Level III-therapeutic.

Re-evaluating consensus and uncertainty among treatment options for early onset scoliosis: a 10-year update.

PURPOSE: Consensus and uncertainty in early onset scoliosis (EOS) treatment were evaluated in 2010. It is currently unknown how treatment preferences have evolved over the past decade. The purpose of this study was to re-evaluate consensus and uncertainty among treatment options for EOS patients to understand how they compare to 10 years ago. METHODS: 11 pediatric spinal surgeons (similar participants as in 2010) were invited to complete a survey of 315 idiopathic and neuromuscular EOS cases (same cases as in 2010). Treatment options included the following: conservative management, distraction-based methods, growth guidance/modulation, and arthrodesis. Consensus was defined as ≥ 70% agreement, and uncertainty was < 70%. Associations between case characteristics and consensus for treatments were assessed via chi-squared and multiple regression analyses. Case characteristics associated with uncertainty were described. RESULTS: Eleven surgeons [31.7 ± 7.8 years of experience] in the original 2010 cohort completed the survey. Consensus for conservative management was found in idiopathic patients aged ≤ 3, whereas in 2010, some of these cases were selected for surgery. There is currently consensus for casting idiopathic patients aged 1 or 2 with moderate curves, whereas in 2010, there was uncertainty between casting and bracing. Among neuromuscular cases with consensus for surgery, arthrodesis was chosen for patients aged 9 with larger curves. CONCLUSION: Presently, preferences for conservative management have increased in comparison to 2010, and casting appears to be preferred over bracing in select infantile cases. Future research efforts with higher levels-of-evidence should be devoted to elucidate the areas of uncertainty to improve care in the EOS population. LEVEL OF EVIDENCE: Level V.

Evaluating the Efficacy of Rib-to-pelvis Growth-friendly Surgery for the Treatment of Non-ambulatory Early-Onset Scoliosis Myelomeningocele Patients.

INTRODUCTION: Early-onset scoliosis (EOS) is a well-known orthopaedic manifestation in patients with myelomeningocele. The rib-based growing system (RBGS) has been proposed as an alternative for these individuals because of the poor outcomes with traditional surgical techniques. We aimed to describe the effect of RBGS in patients with nonambulatory EOS myelomeningocele. METHODS: We retrospectively reviewed the Pediatric Spine Study Group Multicenter Database for all patients with nonambulatory EOS myelomeningocele treated with RBGS from 2004 to 2019. Demographics, surgical data, radiographic findings, and postoperative complications were obtained. The quality-of-life parameters were assessed postoperatively using the Early-onset Scoliosis Questionnaire-24. RESULTS: Thirty patients (18 women; 60%) were patients with nonambulatory EOS myelomeningocele treated with RBGS. The mean age at the initial surgery was 5.3 years. The thoracic (T1-T12) spine height showed a significant increase from initial surgery to the most recent follow-up (P < 0.001). Spine (T1-S1) height was also significantly increased (P < 0.001). The postoperative complication rate was 87%. The Early-onset Scoliosis Questionnaire-24 demonstrates significant improvements in the quality-of-life scores (P = 0.037). CONCLUSION: This study demonstrated that RBGS could improve the reported quality-of-life scores in patients with nonambulatory EOS myelomeningocele when assessed with an EOS-oriented tool. Moreover, we confirmed the ability of RBGS to hold or even correct spinal deformity.

Mortality in Early-Onset Scoliosis During the Growth-friendly Surgery Era.

INTRODUCTION: Early-onset scoliosis (EOS) is a spinal deformity that occurs in patients 9 years of age or younger. Severe deformity may result in thoracic insufficiency, respiratory failure, and premature death. The purpose of this study is to describe the modern-day natural history of mortality in patients with EOS. METHODS: The multicenter Pediatric Spine Study Group database was queried for all patients with EOS who are deceased, without exclusion. Demographics, underlying diagnoses, EOS etiology, operative and nonoperative treatments or observation, complications, and date of death were retrieved. Descriptive statistics and survival analysis with Kaplan-Meier curves were performed. RESULTS: There were 130/8009 patients identified as deceased for a registry mortality rate of 16 per 1000 patients. The mean age at death was 10.6 years (range: 1.0 to 30.2 y) and the most common EOS etiology was neuromuscular (73/130, 56.2%; P<0.001). Deceased patients were more likely be treated operatively than nonoperatively or observed (P<0.001). The mean age of death for patients treated operatively (12.3 y) was older than those treated nonoperatively (7.0 y) or observed (6.3 y) (P<0.001) despite a larger deformity and similar index visit body mass index and ventilation requirements. Kaplan-Meier analysis confirmed an increased survival time in patients with a history of any spine operation compared with patients without a history of spine operation (P<0.0001). Operatively treated patients experienced a median of 3.0 complications from diagnosis to death. Overall, cardiopulmonary related complications were the most common (129/271, 47.6%; P<0.001), followed by implant-related (57/271, 21.0%) and wound-related (26/271, 9.6%). The primary cause of death was identified for 78/130 (60.0%) patients, of which 57/78 (73.1%) were cardiopulmonary related. CONCLUSIONS: This study represents the largest collection of EOS mortality to date, providing surgeons with a modern-day examination of the effects of surgical intervention to better council patients and families. Both fatal and nonfatal complications in children with EOS are most likely to involve the cardiopulmonary system. LEVEL OF EVIDENCE: Level IV-therapeutic.

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

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

No Difference in the Rates of Unplanned Return to the Operating Room Between Magnetically Controlled Growing Rods and Traditional Growth Friendly Surgery for Children With Cerebral Palsy.

BACKGROUND: Early-onset scoliosis (EOS) is common in children with cerebral palsy (CP). The effectiveness of magnetically controlled growing rods (MCGR) and the risk for unplanned return to the operating room (UPROR) remain to be studied in this patient population. The primary outcome of this study was to examine the frequency of UPROR between MCGRs as compared with traditional growth friendly (TGF) surgeries for children with EOS secondary to CP. METHODS: Patients with EOS secondary to CP were prospectively identified from an international database, with data retrospectively analyzed. Scoliosis, kyphosis, T1-S1, and T1-T12 height were measured preoperation, immediate postoperation, and at minimum 2-year follow-up. The risk and etiology of UPRORs were compared between MCGR and TGF. RESULTS: Of the 120 patients that met inclusion criteria, 86 received TGF (age 7.5±0. 1.8 y; mean follow-up 7.0±2.9 y) and 34 received MCGR (age 7.1±2.2 y, mean follow-up 2.8±0.0.5 y). Compared with TGF, MCGR resulted in significant improvements in maintenance of scoliosis (P=0.007). At final follow-up, UPRORs were 8 of 34 patients (24%) for MCGR and 37 of 86 patients (43%) for TGF (P=0.05). To minimize the influence of follow-up period, UPRORs within the first 2 years postoperation were evaluated: MCGR (7 of 34 patients, 21%) versus TGF (20 of 86 patients, 23%; P=0.75). Within the first 2 years, etiology of UPROR as a percentage of all patients per group were deep infection (13% TGF, 6% MCGR), implant failure/migration (12% TGF, 9% MCGR), dehiscence (4% TGF, 3% MCGR), and superficial infection (4% TGF, 3% MCGR). The most common etiology of UPROR for TGF was deep infection and for MCGR was implant failure/migration. CONCLUSION: For patients with EOS secondary to CP, there was no difference in the risk of UPROR within the first 2 years postoperatively whether treated with TGF surgery or with MCGRs (23% TGF, 21% MCGR). LEVEL OF EVIDENCE: Level III-retrospective cohort, therapeutic study.

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.

BACKGROUND: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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 OF EVIDENCE: Level II. This is a multicenter retrospective cohort study investigating the effect of UPROR on HRQoL (prognostic study).

How low can you go? Implant density in posterior spinal fusion converted from growing constructs for early onset scoliosis.

STUDY DESIGN: Retrospective, multicenter comparative. OBJECTIVES: Our purpose was to compare early onset scoliosis (EOS) patients treated with ultra-low, low, and high implant density constructs when undergoing conversion to definitive fusion. Larson et al. demonstrated that implant density (ID) at fusion does not correlate with outcomes in the treatment of adolescent idiopathic scoliosis, but did not address growth-friendly graduates. METHODS: EOS patients treated with growth-friendly constructs converted to fusion between 2000 and 2017 were reviewed from a multicenter database. ID was defined as number of pedicle screws, hooks, and sublaminar/bands per level fused. Patients were divided into ultra-low ID (< 1.3), low (≥ 1.3 and < 1.6), and high ID (≥ 1.6). EXCLUSION CRITERIA: < 2 years follow-up from fusion or inadequate radiographs. RESULTS: A total of 152 patients met inclusion criteria with 39 (26%) patients in the high ID group, 33 (22%) patients in the low ID group, and 80 (52%) in the ultra-low ID group. Groups were similar in operative time (p = 0.61), pre-fusion major curve (p = 0.71), mean number of levels fused (p = 0.58), clinical follow-up (p = 0.30), and radiographic follow-up (p = 0.90). Patients in the low ID group (11.6 ± 1.5 years) were slightly younger at the time of definitive fusion than patients in the ultra-low ID group (12.9 ± 2.2 years) and high ID group (12.5 ± 1.7 years) (p = 0.009). There was significantly more blood loss in the high ID group than the other two groups (high ID: 946.8 ± 606.0 mL vs. low ID: 733.9 ± 434.5 mL and ultra-low ID: 617.4 ± 517.2 mL; p = 0.01), but there was no significant difference with regard to percent of total blood volume lost (high ID: 59.3 ± 48.7% vs. low ID: 54.5 ± 37.5% vs. ultra-low ID: 51.7 ± 54.9%; p = 0.78). There was a difference in initial improvement in major curve between the groups (high ID: 21.6° vs. low ID: 18.0° vs. ultra-low ID: 12.6°; p = 0.01). However, during post-fusion follow-up, correction decreased 7.1° in the high ID group, 2.6 in the low ID group, and 2.8 in the ultra-low ID group (p = 0.19). At final follow-up, major curve correction from pre-fusion was similar between groups (high ID: 14.5° vs. low ID: 15.5° vs. ultra-low ID: 9.7°, p = 0.14). At final follow-up, there was no difference in T1-T12 length gain (p = 0.85), T1-S1 length gain (p = 0.68), coronal balance (p = 0.56), or sagittal balance (p = 0.71). The revision rate was significantly higher in the ultra-low ID group (13.8%; 11/80) versus the high ID group (2/39; 5.1%) and low ID group (0/33; 0%) (p = 0.04). CONCLUSIONS: Although an ID < 1.3 in growth-friendly graduates produces similar outcomes with regard to curve correction and spinal length gain as low and high ID, this study suggests that an ID < 1.3 is associated with an increased revision rate. LEVEL OF EVIDENCE: III.

Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.

BACKGROUND: There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population. METHODS: A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation. RESULTS: Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (±4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced ≥6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following. CONCLUSIONS: This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.

Comparing health-related quality of life and burden of care between early-onset scoliosis patients treated with magnetically controlled growing rods and traditional growing rods: a multicenter study.

STUDY DESIGN: Multicenter retrospective cohort study. OBJECTIVES: To compare pre-operative and post-operative EOSQ-24 scores in magnetically controlled growing rods (MCGR) and traditional growing rod (TGR) patients. Since the introduction of MCGR, early-onset scoliosis patients have been afforded a reduction in the number of surgeries compared to the TGR technique. However, little is known about (health-related quality of life) and burden of care outcomes between these surgical techniques. METHODS: This is a retrospective cohort study using a multicenter registry on patients with EOS undergoing MCGR or TGR between 2008 and 2017. The EOSQ-24 was administered at preoperative and postoperative 2-year assessments. The EOSQ-24 scores were compared between MCGR and TGR as well as preoperatively and postoperatively within each procedure. RESULTS: 110 patients were analyzed in this study (TGR, N = 32; MCGR, N = 78). There were no significant differences in preoperative age, gender, etiology, main coronal curve or maximum kyphosis between TGR and MCGR groups. Patients with TGR had averaged 3.9 surgical lengthenings and MCGR had averaged 7.7 non-invasive lengthenings by the 2-year follow-up. When changes in preoperative to postoperative scores were compared, MCGR had more improvements in pain, emotion, child satisfaction and parent satisfaction than TGR although there were no statistical significance. When analyzed separately, MCGR cohort had improvement in scores for all four domains and four sub-domains; while, TGR cohort only had improvement in financial burden domain and pulmonary function sub-domain. CONCLUSION: Although there was no statistical significance, the improvement in pain, emotion and satisfaction scores was larger in MCGR than TGR. Since these areas can be influenced more by mental well-being than other sub-domains, the results may prove our hypothesis that compared to TGR, MCGR with reduced number of surgeries have better psychosocial effects. LEVEL OF EVIDENCE: III.

Impact of growth friendly interventions on spine and pulmonary outcomes of patients with spinal muscular atrophy.

PURPOSE: Patients with spinal muscular atrophy (SMA) are often treated with growth friendly devices such as vertical expandable prosthetic titanium rib(VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, thorax morphology and pulmonary outcomes. METHODS: This is a retrospective analysis of prospectively collected data. We included patients with chest wall deformity and scoliosis secondary to SMA who were treated with growth friendly interventions and had two-year follow-up. Descriptive statistics and univariate analyses were performed. RESULTS: This study included 66 patients (25% MCGR, 73% VEPTR, 2% unknown). Approximately 23% of constructs included CWS. The average Cobb angle improved from 67° (SD: 27°) to 50° (SD: 26°) at 2 years in patients with CWS (p = 0.02), and from 59° (SD: 20°) to 46° (SD: 15°) at 2 years in patients without CWS (p < 0.01). Hemithorax height improved in patients treated with and without CWS (p = 0.01), but hemithorax width only improved in patients with CWS (p = 0.01). One patient with CWS and two patients without CWS required additional respiratory support at 2 years. The rates of postoperative complications were not significantly different in patients treated with and without CWS (p = 0.31). CONCLUSIONS: Growth friendly constructs improve spinal deformity and may be effective in altering the progression toward respiratory failure in patients with SMA. Patients treated with CWS have significant improvements in thorax morphology compared to patients without CWS.

Minimum 5-Year Follow-up on Graduates of Growing Spine Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth friendly (GF) surgery for early onset scoliosis, patients undergo spinal fusion or continued observation. This last planned treatment is colloquially called "definitive" treatment, conferring these patients as "graduates" of a growing program. The 5-year radiographic and clinical outcomes of this cohort are unknown. METHODS: An international pediatric spine database was queried for patients from a GF program (spine or rib-based) with minimum 5-year follow-up from last planned surgery (GF or spinal fusion). Radiographs and charts were reviewed for main coronal curve angle and maximum kyphosis as well as occurrence of secondary surgery. RESULTS: Of 580 graduates, 170 (29%) had minimum 5-year follow-up (37% male). Scoliosis etiology was congenital in 41 (24%), idiopathic 36 (21%), neuromuscular 51 (30%), and syndromic 42 (25%). Index surgery consisted of spine-based growing rods in 122 (71%) and rib-based distraction in 48 (29%). Mean age at index surgery was 6.8 years, and patients underwent an average of 5.4 lengthenings over an average of 4.9 years (range, 6 mo to 11 y). Last planned treatment was at an average age of 11.8 years (range, 7 to 17 years). Last planned treatment consisted of spinal fusion in 114 patients, 47 had growing implants maintained, 9 had implants removed. Average follow-up was 7.3 years (range, 5 to 13 y).When compared from postdefinitive treatment to 2-year follow-up, there was noted progression of the coronal curve angle (46±19 to 51±21 degrees, P=0.046) and kyphosis (48±20 to 57±25 degrees, P=0.03). However, between 2 and 5 years, no further progression occurred in the coronal (51±21 to 53±21 degrees, P=0.26) or sagittal (57±25 to 54±28 degrees, P=0.93) planes. When stratified based on etiology, there was no significant coronal curve progression between 2- and 5-year follow-up. When comparing spinal fusion patients to those who had maintenance of their growing construct, there was also no significant curve progression.Thirty-seven (21%) underwent at least 1 (average, 1.7; range, 1 to 7) revision surgery following graduation, and 15 of 37 (41%) underwent 2 or more revision surgeries. Reason for revision was implant revision (either GF or spinal fusion) in 34 patients, and implant removal in 3. On an average, the first revision was 2.5 years after the definitive treatment plan (range, 0.02 to 7.4 y). In total, 15 of 37 (41%) revisions occurred over 2 years following the final decision for treatment plan, and 7 of 37 (19%) occurred 5 or more years after the definitive treatment.Patients who underwent spinal fusion as a definitive treatment strategy were more likely to undergo revision surgery (27%) than patients who had their GF implants maintained (11%) (P=0.04). CONCLUSIONS: Five years following "graduation" from growing surgery for early onset scoliosis, there is progression of curve magnitude in both the coronal and sagittal planes up to 2 years, with no further progression at 5 years. A total of 21% of patients undergo at least 1 revision surgery, and average time to revision surgery is over 2 years from last planned surgery. Risk of revision surgery was higher in patients who underwent a spinal fusion as their definitive treatment strategy. LEVEL EVIDENCE: Level III-retrospective comparative. TYPE OF EVIDENCE: Therapeutic.