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.

Outcomes after completing growth-friendly surgical treatment for early-onset scoliosis in patients with skeletal dysplasia.

Aims: The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes. Methods: We retrospectively identified 20 patients with skeletal dysplasias and 292 with other syndromes (control group) who had completed surgical growth-friendly EOS treatment between 1 January 2000 and 31 December 2018. We compared radiological parameters, complications, and health-related quality of life (HRQoL) at mean follow-up of 8.6 years (SD 3.3) in the dysplasia group and 6.6 years (SD 2.6) in the control group. Results: Mean major curve correction per patient did not differ significantly between the dysplasia group (43%) and the control group (28%; p = 0.087). Mean annual spinal height increase was less in the dysplasia group (9.3 mm (SD 5.1) than in the control group (16 mm (SD 9.2); p < 0.001). Mean annual spinal growth adjusted to patient preoperative standing height during the distraction period was 11% in the dysplasia group and 14% in the control group (p = 0.070). The complication rate was 1.6 times higher (95% confidence interval (CI) 1.3 to 2.0) in the dysplasia group. The following complications were more frequent in the dysplasia group: neurological injury (rate ratio (RR) 5.1 (95% CI 2.3 to 11)), deep surgical site infection (RR 2.2 (95% CI 1.2 to 4.1)), implant-related complications (RR 2.0 (95% CI 1.5 to 2.7)), and unplanned revision (RR 1.8 (95% CI 1.3 to 2.5)). Final fusion did not provide additional spinal height compared with watchful waiting (p = 0.054). There were no significant differences in HRQoL scores between the groups. Conclusion: After growth-friendly EOS treatment, patients with skeletal dysplasias experienced a higher incidence of complications compared to those with other syndromes. Surgical growth-friendly treatment for skeletal dysplasia-associated EOS should be reserved for patients with severe, progressive deformities that are refractory to nonoperative treatment.

Late-presenting dural leak following spine fusion in the pediatric population.

PURPOSE: The purpose is to describe how patients with a late-presenting dural leak (LPDL) after posterior spinal fusion (PSF) was diagnosed and treated at a single institution. METHODS: Of the 1991 patients who underwent a PSF between 2010 and 2018, 6 patients were identified with a clinical course consistent with a potential LPDL. RESULTS: Six patients with median age 16.9 years had onset of headache ranging 1-12 weeks postoperatively (median 6.5 weeks). All six patients presented with positional headache, and half (3/6) presented with emesis. 5/6 patients underwent contrast brain MRI, which demonstrated pachymeningeal enhancement. 4/5 patients with dural enhancement went on to have CT myelogram. Five patients had a CT myelogram, which identified a dural leak in all patients and localized the leak in four of five patients. All patients underwent an epidural blood patch, which resolved the pain in five patients. One patient without relief underwent revision surgery with removal of a medially placed screw and fibrin glue placement resolving symptoms. CONCLUSIONS: Postoperative dural leaks associated with PSF may present in a delayed fashion. The majority of leaks were not associated with screw malposition. In diagnosing patients with suspected LPDL, we suggest brain MRI with contrast as a first step. Most patients with pachymeningeal enhancement shown on contrast brain MRI had dural leaks that were identified through CT myelograms. For patients with a dural leak, if there is no disruption from screws, a blood patch appears to be an effective treatment. LEVEL OF EVIDENCE: IV.

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.

Is spinal height gain associated with rod orientation and the use of cross-links in magnetically controlled growing rods in early-onset scoliosis?

Optimal orientation for magnetically controlled growing rods (MCGRs) is unclear. The objective of this study was to investigate associations of rod orientation with implant-related complications (IRCs) and spinal height gains. Using an international early-onset scoliosis (EOS) database, we retrospectively reviewed 57 patients treated with dual MCGRs from May 2013 to July 2015 with minimum 2-year follow-up. Outcomes of interest were IRCs and left/right rod length gains and thoracic (T1-T12) and spinal (T1-S1) heights. We compared patients with two rods lengthened in the cephalad ( standard; n  = 18) versus opposite ( offset; n  = 39) directions. Groups did not differ in age, sex, BMI, duration of follow-up, EOS cause, ambulatory status, primary curve magnitude, baseline thoracic height, or number of distractions/year. We compared patients whose constructs used ≥1 cross-link (CL group; n  = 22) versus no CLs (NCL group; n  = 35), analyzing thoracic height gains per distraction ( α  = 0.05). Offset and standard groups did not differ in left or right rod length gains overall or per year or in thoracic or spinal height gain. Per distraction, the CL and NCL groups did not differ significantly in left or right rod length or thoracic or spinal height gain. Complications did not differ significantly between rod orientation groups or between CL groups. MCGR orientation and presence of cross-links were not associated with differences in rod length gain, thoracic height, spinal height, or IRCs at 2-year follow-up. Surgeons should feel comfortable using either MCGR orientation. Level of evidence: 3, retrospective.

Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.

BACKGROUND: Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1. METHODS: Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs. RESULTS: Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs. CONCLUSION: We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

Quantitative Sensory Changes Following Posterior Spinal Fusion to Treat Adolescent Idiopathic Scoliosis.

BACKGROUND: Sensory changes surrounding the incision frequently develop after posterior spinal fusion (PSF) to treat adolescent idiopathic scoliosis (AIS). Anecdotally, patients may experience sensory changes on the chest wall. Such postsurgical sensory changes are not well described quantitatively. This study aims to evaluate the presence, intensity, and duration of mechanical sensory changes in AIS patients postoperatively. METHODS: A prospective cohort of AIS patients, 10 to 21 years old, was followed. Quantitative sensory testing (QST) included touch detection threshold [mechanical detection threshold (MDT)] and pain detection threshold (MPT), using VonFrey monofilaments and pinprick stimulators. QST was performed at 3 sites at T6: the right and left chest at the nipple line and adjacent to the incision below the inferior angle of the scapula. QST at the thenar eminence was the control. QST was collected at baseline, 3 days, 1, and 6 months postoperative. RESULTS: Thirty-four patients (21% males; mean age: 14.9 years old; median preoperative curve: 58 degrees) completed all testing. Mean deformity correction was 64% (SD: 10.4). Adjacent to the incision site, MDT was significantly higher compared with baseline at 3 days and 1 month ( P < 0.001) but not at 6 months ( P = 0.19), whereas MPT was significantly higher at 3 days, ( P < 0.001), 1 month ( P < 0.001), and 6 months ( P = 0.001). For the chest wall in all patients, MPT was higher on the left chest at 3 days ( P = 0.04) and on the right chest at 3 days ( P = 0.022) and 1 month ( P = 0.05). For patients with right-sided curves, MDT ( P = 0.01) and MPT ( P = 0.015) overall were significantly higher on the concave side (left) chest postoperatively. CONCLUSIONS: PSF is associated with sensory disturbances that are detectable within days, persist at 1 month, and improve at 6 months postoperatively adjacent to the incision and on the chest wall. We suspect that these sensory changes are transient. Describing postoperative sensory changes will help us better set postoperative expectations for patients undergoing PSF. LEVEL OF EVIDENCE: Level I.

Definition of Tweener: Consensus Among Experts in Treating Early-onset Scoliosis.

BACKGROUND: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population. METHODS: A 3-round survey of surgeons in an international EOS study group was conducted. Surgeons were provided with various patient characteristics and asked if each was part of their definition for Tweener patients. Responses were analyzed for consensus (≥70%), near-consensus (60% to 69%), and no consensus (<60%). RESULTS: Consensus was reached (89% of respondents) for including chronological age in the Tweener definition; 8 to 10 years for females and 9 to 11 years for males. Surgeons agreed for inclusion of Sanders score, particularly Sanders 2 (86.0%). Patients who have reached Sanders 4, postmenarche, or have closed triradiate cartilage should not be considered Tweeners. Bone age range of 8 years and 10 months to 10 years and 10 months for females (12 y for males) could be part of the Tweener definition. CONCLUSIONS: This study suggests that the Tweener definition could be the following: patients with open triradiate cartilage who are not postmenarche and have not reached Sanders 4, and if they have one of the following: Sanders 2 or chronological age 8 to 10 years for females (9 to 11 y for males) or bone age 8 years and 10 months to 10 years and 10 months for females (12 y for males). This definition will allow for more focused and comparative research on this population. LEVEL OF EVIDENCE: Level V-expert opinion.

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.

Early Adherence to Prescribed Brace Wear for Adolescent Idiopathic Scoliosis Is Associated With Future Brace Wear.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: To assess if initial adherence to prescribed brace wear for adolescent idiopathic scoliosis (AIS) predicts future adherence and curve improvement. SUMMARY OF BACKGROUND DATA: AIS bracing can be effective if patients adhere to prescribed brace wear. Previous research has associated age, sex, and brace prescription length with future adherence. We hypothesize that a patient's initial adherence to brace wear may be associated with future adherence and outcomes. MATERIALS AND METHODS: Consecutive AIS patients who met the Scoliosis Research Society (SRS) criteria for bracing from 2015 to 2019 at a single center were reviewed. Patients were stratified into groups based on their adherence during the initial one-month brace wean-in period-adherent patients were defined as wearing the brace >80% of the daily prescribed amount. Brace wear was recorded by a thermosensor and assessed during the wean-in period, six-, 12-, and 24-month postbracing appointments. Statistical testing was conducted to analyze if initial adherence was associated with future adherence, curve change, and bracing success -defined as reaching Risser stage 4 with a Cobb angle <40°. RESULTS: Sixty patients (mean age=12.5 yr) were included, of which the majority were females (83%) with thoracic curves (70%). Thirty-two patients were considered adherent, and this cohort demonstrated improved adherence relative to the nonadherent group at the six-, 12-, and 24-month appointments ( P <0.001). Adherent patients also showed a significant reduction in their scoliosis at the 12-month appointment, unlike nonadherent patients ( P <0.001). Ninety-seven percent of adherent patients achieved bracing success compared with 71% of nonadherent ( P =0.016). Females were more likely to be adherent than males. CONCLUSIONS: Initial adherence to prescribed AIS brace wear was associated with future adherence, bracing success, and curve improvement. Early recognition of nonadherence may offer an opportunity for supportive intervention to improve brace wear behavior.

Trends in the Utilization of Implants in Index Procedures for Early Onset Scoliosis From the Pediatric Spine Study Group.

INTRODUCTION: Because of the relative rarity of Early Onset Scoliosis (EOS) cases, patient registries were developed to combine clinical information from multiple institutions to maximize patient care and outcomes. This study examines the history and trends regarding the use of growth-friendly devices for index surgical procedures in EOS patients within the Pediatric Spine Study Group database. METHODS: All index growth-friendly implants were queried from registry inception until October 2020. EOS etiology, device/implant type, and geographic area/institution for each procedure were recorded. RESULTS: From 1994 to 2020, 2786 patients underwent index surgery at a mean age of 6.2±2.9 years. There were 908 traditional growing rods (TGR) (32.3%), 922 vertical expandable prosthetic titanium rib devices (VEPTR) (33.1%), 5 hybrid VEPTR/TGR (0.18%), and 951 magnetically controlled growing rods (MCGR) (34.2%) index implants. Fifty-six different institutions reported an index implant, and 5 accounted for 823 (30%) of the cases during the study period. Institutions in the Northeast accounted for more index implants than other regions of the United States. There was a 40% increase in index implant insertions annually when comparing 1994 (3 implants/1 center) to 2018 (234 implants/56 centers), ( P <0.001). Beginning in 2009, there was a 90.9% decrease in the number of TGR/VEPTR procedures (2009: 156 implants/32 centers; 2019: 22 implants/49 centers P =0.001), and a 479% increase in MCGR (2009: 1 implant/1 center; 2018: 197 implants/34 centers ( P =0.005). The overall number of growth-friendly index procedures performed in 2019 (150/49 centers) decreased 34.5% when compared to 2018 (234/48 centers). CONCLUSION: The number of growth-friendly implants reported in the Pediatric Spine Study Group registry as the initial surgical management of EOS increased markedly over the past 20 years. MCGR is currently the predominant type of device utilized for index surgical procedures by group members, surpassing the use of VEPTR and TGR in 2014. There was a significant decrease in index growth-friendly procedures in 2019 compared to 2018. LEVEL OF EVIDENCE: Level IV.

Bracing for juvenile idiopathic scoliosis: retrospective review from bracing to skeletal maturity.

BACKGROUND: Juvenile idiopathic scoliosis (JIS) outcomes with brace treatment are limited with poorly described bracing protocols. Between 49 and 100% of children with JIS will progress to surgery, however, young age, long follow-up, and varying treatment methods make studying this population difficult. The purpose of this study is to report the outcomes of bracing in JIS treated with a Boston brace™ and identify risk factors for progression and surgical intervention. METHODS: This is a single-center retrospective review of 175 patients with JIS who initiated brace treatment between the age of 4 and 9 years. A cohort of 140 children reached skeletal maturity; 91 children had surgery or at least 2 year follow-up after brace completion. Standard in-brace protocol for scoliosis 320° was a Boston brace for 18-20 h/day after MRI (n = 82). Family history, MRI abnormalities, comorbidities, curve type, curve magnitude, bracing duration, number of braces, compliance by report, and surgical interventions were recorded. RESULTS: Children were average 7.9 years old (range 4.1-9.8) at the initiation of bracing. The Boston brace™ was prescribed in 82 patients and nine used night bending brace. Mid-thoracic curves (53%) was the most frequent deformity. Maximum curve at presentation was on average 30 ± 9 degrees, in-brace curve angle was 16 ± 8 degrees, and in-brace correction was 58 ± 24 percent. Patients were braced an average of 4.6 ± 1.9 years. 61/91 (67%) went on to posterior spinal fusion at 13.3 ± 2.1 (range 9.3-20.9) years and curve magnitude of 61 ± 12 degrees. Of those that underwent surgery, 49/55 (86%) progressed > 10°, 6/55 (11%) stabilized within 10°, and 0/55 (0%) improved > 10° with brace wear. No children underwent growth-friendly posterior instrumentation. Of the 28 who did not have surgical correction, 3 (11%) progressed > 10°, 13/28 (46%) stabilized within 10°, and 12/28 (43%) improved > 10° with brace wear. CONCLUSIONS: This large series of JIS patients with bracing followed to skeletal maturity with long-term follow-up. Surgery was avoided in 33% of children with minimal to no progression, and no child underwent posterior growth-friendly constructs. Risk factors of needing surgery were noncompliance and larger curves at presentation.

Contraindications to magnetically controlled growing rods: consensus among experts in treating early onset scoliosis.

PURPOSE: The purpose of this study was to describe contraindications to the magnetically controlled growing rod (MCGR) in patients with early onset scoliosis (EOS) by establishing consensus amongst expert surgeons who treat these patients frequently. METHODS: Nine pediatric spine surgeons from an international EOS study group participated in semi-structured interviews via email to identify factors that influence decision making in the use of MCGR. A 39-question survey was then developed to specify these factors as contraindications for MCGR-these included patient age and size, etiology, medical comorbidities, coronal and sagittal curve profiles, and skin and soft tissue characteristics. Pediatric spine surgeons from the EOS international study group were invited to complete the survey. A second 29-item survey was created to determine details and clarify results from the first survey. Responses were analyzed for consensus (> 70%), near consensus (60-69%), and no consensus/variability (< 60%) for MCGR contraindication. RESULTS: 56 surgeons of 173 invited (32%) completed the first survey, and 64 (37%) completed the second survey. Responders had a mean of over 15 years in practice (range 1-45) with over 6 years of experience with using MCGR (range 2-12). 71.4% of respondents agreed that patient size characteristics should be considered as contraindications, including BMI (81.3%) and spinal height (84.4%), although a specific BMI range or a specific minimum spinal height were not agreed upon. Among surgeons who agreed that skin and soft tissue problems were contraindications (78.6%), insufficient soft tissue (98%) and skin (89%) to cover MCGR were specified. Among surgeons who reported curve stiffness as a contraindication (85.9%), there was agreement that this curve stiffness should be defined by clinical evaluation (78.2%) and by traction films (72.3%). Among surgeons who reported sagittal curve characteristics as contraindications, hyperkyphosis (95.3%) and sagittal curve apex above T3 (70%) were specified. Surgeons who indicated the need for repetitive MRI as a contraindication (79.7%) agreed that image quality (72.9%) and not patient safety (13.6%) was the concern. In the entire cohort, consensus was not achieved on the following factors: patient age (57.4%), medical comorbidities (46.4%), etiology (53.6%), and coronal curve characteristics (58.9%). CONCLUSION: Surgeon consensus suggests that MCGR should be avoided in patients who have insufficient spinal height to accommodate the MCGR, have potential skin and soft tissue inadequacy, have too stiff a spinal curve, have too much kyphosis, and require repetitive MRI, particularly of the spine. Future data-driven studies using this framework are warranted to generate more specific criteria (e.g. specific degrees of kyphosis) to facilitate clinical decision making for EOS patients. LEVEL OF EVIDENCE: Level V-expert opinion.

Spinal Fusion in Pediatric Patients With Low Bone Density: Defining the Value of DXA.

BACKGROUND: Children with medical complexity are at increased risk of low bone mineral density (BMD) and complications after spinal fusion compared with idiopathic scoliosis patients. Our aim was to compare treatments and outcomes of children with medical complexity undergoing spinal fusion in those who had dual-energy x-ray absorptiometry (DXA) scans versus those who did not in an effort to standardize the workup of these patients before undergoing spinal surgery. METHODS: We conducted a retrospective review of patients with low BMD who underwent spinal fusion at a tertiary care pediatric hospital between 2004 and 2016. We consulted with a pediatric endocrinologist to create standard definitions for low BMD to classify each subject. Regardless of DXA status, all patients were given a clinical diagnosis of osteoporosis [at least 2 long bone or 1 vertebral pathologic fracture(s)], osteopenia (stated on radiograph or by the physician), or clinically low bone density belonging to neither category. The last classification was used for patients whose clinicians had documented low bone density not meeting the criteria for osteoporosis or osteopenia. Fifty-nine patients met the criteria, and 314 were excluded for insufficient follow-up and/or not meeting a diagnosis definition. BMD Z -scores compare bone density ascertained by DXA to an age-matched and sex-matched average. Patients who had a DXA scan were also given a DXA diagnosis of low bone density (≤-2 SD), slightly low bone density (-1.0 to -1.9 SD), or neither (>-1.0 SD) based on the lowest BMD Z -score recorded. RESULTS: Fifty-nine patients were analyzed. Fifty-four percent had at least 1 DXA scan preoperatively. Eighty-one percent of DXA patients received some form of treatment compared with 52% of non-DXA patients ( P =0.03). CONCLUSIONS: Patients referred for DXA scans were more likely to be treated for low BMD, although there is no standardized system in place to determine which patients should get scans. Our research highlights the need to implement clinical protocols to optimize bone health preoperatively. LEVEL OF EVIDENCE: Level II-retrospective prognostic study.

Posterior-only Resection of Single Hemivertebrae With 2-Level Versus >2-Level Fusion: Can We Improve Outcomes?

BACKGROUND: The outcomes of congenital scoliosis (CS) patients undergoing hemivertebra (HV) resection surgery with a 2-level fusion versus a >2-level fusion are unclear. We hypothesized that CS patients undergoing HV resection and a >2-level fusion have decreased curve progression and reoperation rates compared with 2-level fusions. METHODS: Retrospective review of prospectively collected data from a multicenter scoliosis database. Fifty-three CS patients (average age 4.5, range 1.2 to 10.9 y) at index surgery were included. Radiographic and surgical parameters, complications, as well as revision surgery rates were tracked at a minimum of 2-year follow-up. RESULTS: Twenty-six patients had a 2-level fusion while 27 patients had a >2-level fusion with similar age and body mass index between groups. The HV was located in the lumbar spine for 69% (18/26) 2-level fusions and 30% (8/27) >2-level fusions ( P =0.006). Segmental HV scoliosis curve was smaller in 2-level fusions compared to >2-level fusions preoperatively (38 vs. 50 degrees, P =0.016) and at follow-up (25 vs. 34 degrees, P =0.038). Preoperative T2-T12 (28 vs. 41 degrees, P =0.013) and segmental kyphosis (11 vs. 23 degrees, P =0.046) were smaller in 2-level fusions, but did not differ significantly at postoperative follow-up (32 vs. 39 degrees, P =0.22; 13 vs. 11 degrees, P =0.64, respectively). Furthermore, the 2 groups did not significantly differ in terms of surgical complications (27% vs. 22%, P =0.69; 2-level fusion vs. >2-level fusion, respectively), unplanned revision surgery rate (23% vs. 22%, 0.94), growing rod placement or extension of spinal fusion (15% vs. 15%, P =0.95), or health-related quality of life per the EOS-Questionnaire 24 (EOSQ-24). Comparison of patients with or without the need for growing rod placement or posterior spinal fusion revealed no significant differences in all parameters analyzed. CONCLUSIONS: Two-level and >2-level fusions can control congenital curves successfully. No differences existed in curve correction, proximal junctional kyphosis or complications between short and long-level fusion after HV resection. Both short and long level fusions are viable options and generate similar risk of revision. The decision should be individualized by patient and surgeon.

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.

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

PURPOSE: 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. METHODS: 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. RESULTS: Seven patients were analyzed (3 males, 4 females). Patients were at a median age of 2.8 years at initial surgery with median follow-up 3.6 years (range 2.0-12 years) 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 5.0 years (range, 2.6-10.4 years) 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. CONCLUSIONS: 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.

Preoperative hematocrit and platelet count are associated with blood loss during spinal fusion for children with neuromuscular scoliosis.

AIM: To assess the relationship of preoperative hematology laboratory results with intraoperative estimated blood loss and transfusion volumes during posterior spinal fusion for pediatric neuromuscular scoliosis. METHODS: Retrospective chart review of 179 children with neuromuscular scoliosis undergoing spinal fusion at a tertiary children's hospital between 2012 and 2017. The main outcome measure was estimated blood loss. Secondary outcomes were volumes of packed red blood cells, fresh frozen plasma, and platelets transfused intraoperatively. Independent variables were preoperative blood counts, coagulation studies, and demographic and surgical characteristics. Relationships between estimated blood loss, transfusion volumes, and independent variables were assessed using bivariable analyses. Classification and Regression Trees were used to identify variables most strongly correlated with outcomes. RESULTS: In bivariable analyses, increased estimated blood loss was significantly associated with higher preoperative hematocrit and lower preoperative platelet count but not with abnormal coagulation studies. Preoperative laboratory results were not associated with intraoperative transfusion volumes. In Classification and Regression Trees analysis, binary splits associated with the largest increase in estimated blood loss were hematocrit ≥44% vs. <44% and platelets ≥308 vs. <308 × 109/L. CONCLUSIONS: Preoperative blood counts may identify patients at risk of increased bleeding, though do not predict intraoperative transfusion requirements. Abnormal coagulation studies often prompted preoperative intervention but were not associated with increased intraoperative bleeding or transfusion needs.

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).