What is the role of plastic surgery for incisional closures in pediatric spine surgery? Results from a pediatric spine study group survey.

Current best practice guidelines recommend a plastics-style multilayer wound closure for high-risk pediatric spine surgery. However, plastic surgery closure of spinal incisions remains controversial. This study investigates surgeon perceptions and practice patterns regarding plastic surgery multilayered closure (PMC) in pediatric spine surgery. All surgeons in an international pediatric spine study group received a 30-question survey assessing incisional closure practices, frequency of plastic surgery collaboration, and drain management. Relationship to practice size, setting, geographic region, and individual diagnoses were analyzed. 87/178 (49%) surgeons responded from 79% of participating sites. Plastics utilization rates differed by diagnosis: neuromuscular scoliosis 16.9%, early onset scoliosis 7.8%, adolescent idiopathic scoliosis 2.8% (P < 0.0001). Plastics were used more for early onset scoliosis [odds ratio (OR) 18.5, 95% confidence interval (CI): 8.5, 40.2; P < 0.001] and neuromuscular scoliosis [OR 29.2 (12.2, 69.9); P < 0.001] than adolescent idiopathic scoliosis. Plastics use was unrelated to practice size, setting, or geographic region (P ≥ 0.09). Respondents used plastics more often for spina bifida and underweight patients compared to all other indications (P < 0.001). Compared to orthopaedic management, drains were utilized more often by plastic surgery (85 vs. 21%, P = 0.06) and for longer durations (P = 0.001). Eighty-nine percent of surgeons felt plastics increased operative time (58 ±â€…37 min), and 34% felt it increased length of hospitalization. Surgeons who routinely utilize plastics were more likely to believe PMC decreases wound complications (P = 0.007). The perceived benefit of plastic surgery varies, highlighting equipoise among pediatric spine surgeons. An evidence-based guideline is needed to optimize utilization of plastics in pediatric spine surgery.

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

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

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.

Hip Surgery is Not More Painful Than Spine Surgery in Children With Cerebral Palsy.

BACKGROUND: Posterior spinal fusion (PSF) and hip reconstruction are commonly indicated surgeries in children with cerebral palsy (CP), particularly those functioning at GMFCS levels IV and V. These are large and often painful procedures, and previous literature suggests that hip surgery is more painful than spine surgery in this patient population. The purpose of this study is to investigate pain scores and opioid use following hip and spine surgery in a large cohort of children with CP, including many patients who have undergone both types of surgery. METHODS: A retrospective chart review was performed to identify children with CP who underwent hip reconstruction and/or PSF at a tertiary children's hospital between 2004 and 2022. Charts were reviewed for demographic data, pain scores, pain medication usage, duration of hospital stay, and complications. RESULTS: Data were collected for 200 patients (101 male, 99 female) who met inclusion criteria. Eighty-seven patients underwent hip reconstruction, 62 spinal fusion, and 51 both hip and spine surgery asynchronously. Median (interquartile range) age at the time of surgery was significantly older for spinal fusion compared with hip surgery [13.1 (4.9) vs. 8.1 (5.7) y, P<0.0001]. Length of stay was significantly longer after PSF, with a median of 6 (4) days compared with 2 (1) days after hip surgery (P<0.0001). Both maximum and average daily pain scores were similar following hip and spine surgery, with the exception that average pain scores for hip surgery were slightly higher on postoperative day 1, hip=1.73 vs. spine=1.0 (P<0.0001). The amount of opioids used, expressed as morphine milligram equivalents (MME)/kg were similar in the hip and spine surgery groups; however, it was significantly lower in the hip surgery group on postoperative day 0, hip=0.06 versus spine=0.17 (P<0.0001). For the 51 patients who underwent both hip and spine surgery, the amount of opioids used mirrored that for the entire group (similar MME/kg, though only statistically significantly less on POD 0 and 3), and pain scores were not significantly different between the 2 groups except in 2 circumstances. The 2 exceptions in these 51 patients both demonstrated lower pain scores in patients after hip surgery, including lower maximum pain scores on POD 1 (P=0.041), and lower average pain scores on POD3 (P=0.043). CONCLUSIONS: This is the largest series to date comparing postoperative pain in children with CP after hip and spine surgery, including 51 of 200 patients who underwent both types of surgery. The results of this study demonstrate that hip surgery is not more painful than spine surgery in children with CP, and conflict with the traditional belief that hip surgery is more painful. This is important information for health care providers when counseling patients and families regarding these surgeries in children with CP. LEVEL OF EVIDENCE: Level 3.

Opioid consumption before discharge predicts outpatient opioid use in adolescents undergoing surgery.

BACKGROUND: The majority of adolescents undergoing surgery report unused prescription opioids after surgery, increasing the risk of diversion, misuse, and addiction. Adult studies have demonstrated that opioid use 24 hours before discharge corresponds with opioid use at home. We hypothesized that inpatient opioid consumption is associated with outpatient opioid use in adolescents. METHODS: Adolescents aged 13-20 years undergoing elective surgery associated with an opioid prescription were prospectively recruited. Parent-adolescent dyads were surveyed preoperatively to assess sociodemographics, health literacy, and baseline substance use, and opioid use was measured at 30- and 90-days postoperatively. Medical records were reviewed to calculate cumulative opioid use during hospitalization. Inpatient and postoperative opioid use was converted to oral morphine equivalents. Adjusting for age, sex, race, health literacy, alcohol use, pain score, and surgery, multivariable linear regression identified factors associated with outpatient oral morphine equivalent use 90 days postoperatively. RESULTS: Overall, 103 adolescents were enrolled. Median oral morphine equivalents used from 24 and 48 hours before discharge and throughout the hospitalization were 30.8 (interquartile range:11.7-45.0), 67.5 (interquartile range:37.5-94.3), and 97.5 (interquartile range:18.0-152.7), respectively. Regression analysis demonstrated that adolescent-reported pain at discharge (P = .028) and cumulative oral morphine equivalents used 24 hours (P < .001) and 48 hours (P = .003) before discharge were significantly associated with postoperative oral morphine equivalents use at home. Oral morphine equivalents consumption 24 hours before discharge estimated cumulative oral morphine equivalents use 90 days postoperatively at a 1-to-5 ratio in 94.1% of patients. CONCLUSION: For adolescents undergoing surgery, patient-reported pain at discharge and oral morphine equivalents administered 24 hours before discharge are associated with cumulative outpatient opioid use. Tailoring outpatient prescriptions to total oral morphine equivalent consumption 24 hours before discharge could reduce excess opioid prescribing.

How do implants overlying the spine influence "The Law of Diminishing Returns" in early-onset scoliosis patients?

PURPOSE: The "law of diminishing returns" (LODR) in early-onset scoliosis (EOS) is well-known. We hypothesized that previously observed variations between constructs may be related to the lateral distance that each construct lies from the spine. We therefore sought to determine whether the curve magnitude improvement and spinal length gains for distraction-based constructs in EOS are positively correlated with the collinearity of the spine and the convex-sided implant on posteroanterior radiographs. METHODS: A prospectively-collected, multicenter EOS registry was queried for all patients who underwent non-fusion, distraction-based instrumentation surgery. Post-index radiographs were graded from 1 to 5 based on amount of overlap between the convex-sided rod and the apical vertebra. Grade 1: convex rod is lateral to convex-sided pedicle; Grade 2: overlaps the convex-sided pedicle; Grade 3: lies between pedicles; Grade 4: overlaps concave-sided pedicle; Grade 5: medial to concave-sided pedicle. ANOVA assessed the correlations between post-index overlap grade and change in (a) curve magnitude and (b) T1-T12 height. Multivariable regression modeling further assessed these associations. RESULTS: 284 patients met all selection criteria and were included. On ANOVA, post-index grade was associated with curve magnitude (p <0.001) and T1-12 height (p = 0.028) change. Better curve correction and height change were associated with higher grade. On regression modeling, curve correction (R = 0.574) and T1-T12 height change (R = 0.339) remained significantly associated with grade when controlling for time, anchor locations, age, underlying diagnosis, and pre-index curve magnitude. CONCLUSION: More apical overlap by the convex rod was associated with better spinal deformity control and improved height gain. LEVEL OF EVIDENCE III: Therapeutic.

Incidental dural tears during pediatric posterior spinal fusions.

PURPOSE: To characterize the frequency of incidental dural tears in pediatric spine surgery, their treatment, complications, and results of long-term follow-up. METHODS: A retrospective review of all pediatric patients who underwent a posterior spinal fusion (PSF) between 2004-2019 at a tertiary children's hospital was conducted. Electronic medical records were reviewed for patient demographics, intra-operative data, presence of an incidental dural tear, repair method, and patient outcomes. RESULTS: 3043 PSFs were reviewed, with 99 dural tears identified in 94 patients (3.3% overall incidence). Mean follow-up was 35.7 months (range 0.1-142.5). When the cause of the dural tear was specified, 69% occurred during exposure, 5% during pedicle screw placement, 4% during osteotomy, 2% during removal of implants, and 2% during intra-thecal injection of morphine. The rate of dural tears during primary PSF was significantly lower than during revision PSF procedures (2.6% vs. 6.2%, p < 0.05). 86.9% of dural tears were repaired and/or sealed intraoperatively, while 13.1% had spontaneous resolution. Postoperative headaches developed in 13.1% of patients and resolved at a mean of 7.6 days. There was no difference in the incidence of headaches in patients that were ordered bedrest vs. no bedrest (p > 0.99). Postoperative infections occurred in 9.5% of patients and 24.1% patients were identified to have undergone a revision surgery. CONCLUSIONS: Incidence of intra-operative dural tears in pediatric spine surgery is 3.3%. Although complications associated with the dural tear occur, most resolve over time and there were no long-term sequelae in patients with 2 years of follow up. LEVEL OF EVIDENCE: Level IV.

Timing of intraoperative neurophysiological monitoring (IONM) recovery and clinical recovery after termination of pediatric spinal deformity surgery due to loss of IONM signals.

BACKGROUND CONTEXT: Intraoperative neurophysiological monitoring (IONM) is used to reduce the risk of spinal cord injury during pediatric spinal deformity surgery. Significant reduction and/or loss of IONM signals without immediate recovery may lead the surgeon to acutely abort the case. The timing of when monitorable signals return remains largely unknown. PURPOSE: The goal of this study was to investigate the correlation between IONM signal loss, clinical examination, and subsequent normalization of IONM signals after aborted pediatric spinal deformity surgery to help determine when it is safe to return to the operating room. STUDY DESIGN/SETTING: This is a multicenter, multidisciplinary, retrospective study of pediatric patients (<18 years old) undergoing spinal deformity surgery whose surgery was aborted due to a significant reduction or loss of IONM potentials. PATIENT SAMPLE: Sixty-six patients less than 18 years old who underwent spinal deformity surgery that was aborted due to IONM signal loss were enrolled into the study. OUTCOME MEASURES: IONM data, operative reports, and clinical examinations were investigated to determine the relationship between IONM loss, clinical examination, recovery of IONM signals, and clinical outcome. METHODS: Information regarding patient demographics, deformity type, clinical history, neurologic and ambulation status, operative details, IONM information (eg, quality of loss [SSEPs, MEPs], laterality, any recovery of signals, etc.), intraoperative wake-up test, postoperative neurologic exam, postoperative imaging, and time to return to the operating were all collected. All factors were analyzed and compared with univariate and multivariate analysis using appropriate statistical analysis. RESULTS: Sixty-six patients were enrolled with a median age of 13 years [IQR 11-14], and the most common sex was female (42/66, 63.6%). Most patients had idiopathic scoliosis (33/66, 50%). The most common causes of IONM loss were screw placement (27/66, 40.9%) followed by rod correction (19/66, 28.8%). All patients had either complete bilateral (39/66, 59.0%), partial bilateral (10/66, 15.2%) or unilateral (17/66, 25.8%) MEP loss leading to termination of the case. Overall, when patients were returned to the operating room 2 weeks postoperatively, nearly 75% (40/55) had monitorable IONM signals. Univariate analysis demonstrated that bilateral SSEP loss (p=.019), bilateral SSEP and MEP loss (p=.022) and delayed clinical neurologic recovery (p=.008) were significantly associated with having unmonitorable IONM signals at repeat surgery. Multivariate regression analysis demonstrated that delayed clinical neurologic recovery (> 72 hours) was significantly associated with unmonitorable IONM signals when returned to the operating room (p=.006). All patients ultimately made a full neurologic recovery. CONCLUSIONS: In children whose spinal deformity surgery was aborted due to intraoperative IONM loss, there was a strong correlation between combined intraoperative SSEP/MEP loss, the magnitude of IONM loss, the timing of clinical recovery, and the time of electrophysiological IONM recovery. The highest likelihood of having a prolonged postoperative neurological deficit and undetectable IONM signals upon return to the OR occurs with bilateral complete loss of SSEPs and MEPs.

Analysis of three-dimensional spine growth for vertebral body tethering patients at 2 and 5 years post operatively.

PURPOSE: Scoliosis can be treated with vertebral body tethering (VBT) as a motion-sparing procedure. However, the knowledge of how growth is affected by a tether spanning multiple levels is unclear in the literature. Three-dimensional true spine length (3D-TSL) is a validated assessment technique that accounts for the shape of the spine in both the coronal and sagittal planes. This study aimed to assess if 3D-TSL increases over a five-year period after VBT implantation in thoracic curves for idiopathic scoliosis. METHODS: Prospectively collected radiographic data from an international pediatric spine registry was analyzed. Complete radiographic data over three visits (post-operative, 2 years, and 5 years) was available for 53 patients who underwent VBT. RESULTS: The mean age at instrumentation of this cohort was 12.2 (9-15) years. The average number of vertebrae instrumented was 7.3 (SD 0.7). Maximum Cobb angles were 50° pre-op, which improved to 26° post-op (p < 0.001) and was maintained at 5 years (30°; p = 0.543). Instrumented Cobb angle was 22° at 5 years (p < 0.001 vs 5-year maximum Cobb angle). An accentuation was seen in global kyphosis from 29° pre-operative to 41° at 5 years (p < 0.05). The global spine length (T1-S1 3D-TSL) started at 40.6 cm; measured 42.8 cm at 2 years; and 44.0 cm at the final visit (all p < 0.05). At 5 years, patients reached an average T1-S1 length that is comparable to a normal population at maturity. Immediate mean post-operative instrumented 3D-TSL (top of UIV-top of LIV) was 13.8 cm two-year length was 14.3 cm; and five-year length was 14.6 cm (all p < 0.05). The mean growth of 0.09 cm per instrumented level at 2 years was approximately 50% of normal thoracic growth. Patients who grew more than 0.5 cm at 2 years had a significantly lower BMI (17.0 vs 19.0, p < 0.05) and smaller pre-operative scoliosis (48° vs 53°, p < 0.05). Other subgroup analyses were not significant for age, skeletal maturity, Cobb angles or number of spanned vertebras as contributing factors. CONCLUSIONS: This series demonstrates that 3D-TSL increased significantly over the thoracic instrumented levels after VBT surgery for idiopathic scoliosis. This represented approximately 50% of expected normal thoracic growth over 2 years.

Coronal decompensation following thoracic vertebral body tethering in idiopathic scoliosis.

PURPOSE: Post-operative coronal decompensation (CD) continues to be a challenge in the treatment of adolescent idiopathic scoliosis (AIS). CD following selective spinal fusion has been studied. However, there is currently little information regarding CD following Vertebral Body Tethering (VBT). Thus, the goal of this study is to better understand the incidence and risk factors for CD after VBT. METHODS: Retrospective review of a prospective multicenter database was used for analysis. Inclusion criteria were patients undergoing thoracic VBT, a minimum 2-year follow-up, LIV was L1 or above, skeletally immature (Risser ≤ 1), and available preoperative and final follow-up AP and lateral upright radiographs. Radiographic parameters including major and minor Cobb angles, curve type, LIV tilt/translation, L4 tilt, and coronal balance were measured. CD was defined as the distance between C7PL and CSVL > 2 cm. Multiple logistic regression model was used to identify significant predictors of CD. RESULTS: Out of 136 patients undergoing VBT, 94 patients (86 female and 6 male) met the inclusion criteria. The mean age at surgery was 12.1 (9-16) and mean follow-up period was 3.4 years (2-5 years). Major and minor curves, AVR, coronal balance, LIV translation, LIV tilt, L4 tilt were significantly improved after surgery. CD occurred in 11% at final follow-up. Lenke 1A-R (24%) and 1C (26%) had greater incidence of CD compared to 1A-L (4%), 2 (0%), and 3 (0%). LIV selection was not associated with CD. Multivariate logistic regression analysis yielded 1A-R and 1C curves as a predictor of CD with the odds ratio being 17.0. CONCLUSION: CD occurred in 11% of our thoracic VBT patients. Lenke 1A-R and 1C curve types were predictors for CD in patients treated with VBT. There were no other preoperative predictors associated with CD.

Spinal Manifestations of Skeletal Dysplasia: A Practical Guide for Clinical Diagnosis.

Skeletal dysplasias are a group of genetic conditions defined by atypical bone or cartilage growth and development. Skeletal abnormalities include short stature, limb deformity, joint contracture, and spinal deformity. Over 90% of disorders have a known genetic mutation that can definitively determine the diagnosis. As patients may present with a primary spinal concern, a careful clinical and radiographic evaluation can allow the physician to develop a working diagnosis to guide additional evaluation. Spinal manifestations include scoliosis and kyphoscoliosis, cervical instability, cervical kyphosis, thoracolumbar kyphosis, spinal stenosis, and atypical vertebral body morphology. An understanding of the affected conditions, prevalence, and natural history of these radiographic findings aids the orthopaedic surgeon in establishing a diagnosis and guides appropriate orthopaedic care.

Prescription Opioid Use for Adolescents With Neurocognitive Disability Undergoing Surgery: A Pilot Study.

INTRODUCTION: Parents frequently report retaining unused opioid pills following their child's surgery due to fear of untreated postoperative pain. Assessment of pain in adolescents with neurocognitive disability is challenging. We hypothesized that parents of adolescents with neurocognitive disability may report less opioid use and higher opioid pill retention. METHODS: Adolescents (13-20 y) undergoing elective surgery (posterior spinal fusion, hip reconstruction, arthroscopy, tonsillectomy) were prospectively enrolled from a tertiary children's hospital from 2019 to 2020. Only adolescents prescribed opioids at discharge were included. Parents completed a preoperative survey collecting sociodemographic characteristics and two postoperative surveys at 30- and 90-d. Neurocognitive disability was determined at time of enrollment by caregiver report, and included adolescents with cerebral palsy, severe autism spectrum disorder, and discrete syndromes with severe neurocognitive disability. RESULTS: Of 125 parent-adolescent dyads enrolled, 14 had neurocognitive disability. The median number of opioid pills prescribed at discharge did not differ by neurocognitive disability (29, interquartile range {IQR}: 20.0-33.3 versus 30, IQR: 25.0-40.0, P = 0.180). Parents of both groups reported similar cumulative days of opioid use (7.0, IQR: 3.0-21.0 versus 6.0, IQR:3.0-10.0, P = 0.515) and similar number of opioid pills used (4, IQR: 2.0-4.5 versus 12, IQR: 3.5-22.5, P = 0.083). Parents of both groups reported similar numbers of unused opioid pills (17, IQR: 12.5-22.5 versus 19, IQR: 8.0-29.0, P = 0.905) and rates of retention of unused opioids (15.4% versus 23.8%, P = 0.730). CONCLUSIONS: The number of opioid pills prescribed did not differ by neurocognitive disability and parents reported similar opioid use and retention of unused opioid pills. Larger studies are needed to identify opportunities to improve postoperative pain control for children with neurocognitive disability.

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.

Concave and convex growth do not differ over tethered vertebral segments, even with open tri-radiate cartilage.

PURPOSE: To assess the following hypotheses related to vertebral body tethering (VBT): 1. VBT is associated with asymmetric (concave > convex) increases in height over the instrumented vertebra. 2. The instrumented Cobb angle improves following VBT surgery with growth. METHODS: This is a retrospective case series of pediatric patients from a multicenter scoliosis registry treated with VBT between 2013 to 2021. INCLUSION CRITERIA: patients with standing radiographs at < 4 months and ≥ 2 years after surgery. Distances between the superior endplate of the UIV and the inferior endplate of the LIV were measured at the concave corner, mid-point, and convex corner of the endplates. The UIV-LIV angle was recorded. Subgroup analyses included comparing different Risser scores and tri-radiate cartilage (TRC) closed versus open using student t-tests. RESULTS: 83 patients met inclusion criteria (92% female; age at time of surgery 12.5 ± 1.4 years) with mean follow-up time of 3.8 ± 1.4 years. Risser scores at surgery were: 0 (n = 33), 1 (n = 12), 2 (n = 10), 3 (n = 11), 4 (n = 12), and 5 (n = 5). Of the 33 Risser 0 patients, 17 had an open TRC, 16 had a closed TRC. The UIV-LIV distance at concave, middle, and convex points significantly increased from immediate post-op to final-follow-up for Risser 0 patients, but not for Risser 1-5 patients. Increases in UIV-LIV distance were not significantly different between concave, middle, and convex points for all groups. There was no significant improvement or worsening in UIV-LIV angle for any group. CONCLUSION: At a mean of 3.8 years following VBT, 33 Risser 0 patients demonstrated significant growth in the instrumented segment, though there was no difference between concave or convex growth, even for patients with open TRC.

Deformity angular ratio is associated with neuromonitoring changes without a vertebral column resection: spinal deformity is more influential than type of surgery.

PURPOSE: The purpose of this study is to investigate the effect of the deformity angular ratio (DAR) on intra-operative neuromonitoring (IONM) signal changes during posterior spinal fusion (PSF) without vertebral column resection (VCR). METHODS: Retrospective review of severe pediatric spinal deformity patients treated with PSF without VCR or three-column osteotomy from 2008 to 2018. Exclusion criteria were prior instrumentation, lack of IONM, and incomplete radiographic data. Coronal DAR (C-DAR), sagittal DAR (S-DAR), and total DAR (T-DAR) were calculated and compared between patients with IONM signal loss and those without. RESULTS: Two hundred and fifty-three patients met inclusion criteria. Forty-seven of two hundred and fifty-three (19%) patients had IONM signal loss. Intra-operative wake-up test was performed in seven cases; three of seven (43%) had a neurological deficit on wake-up test. All neurological deficits resolved at a mean of 41 days postop. IONM loss was associated with increased kyphosis (p = 0.003) and was not associated with Cobb angle (p = 0.16). S-DAR (p = 0.03) and T-DAR (p = 0.005) were associated with IONM signal loss but C-DAR was not (p = 0.06). Increased incidence of IONM signal loss was seen with S-DAR > 7 (p = 0.02) or T-DAR > 27 (p = 0.02). Twenty-four of ninety-two (26%) patients with S-DAR > 7 had IONM signal loss compared to twenty-three of one hundred and sixty-one (14%) with S-DAR ≤ 7 (OR, 2.1; 95% CI, 1.1-4.0). Seven of sixteen (44%) patients with T-DAR > 27 had signal loss compared to forty of two hundred and thirty-seven (17%) patients with T-DAR ≤ 27 (OR, 3.8; 95% CI, 1.3-10.9). CONCLUSION: Patients with S-DAR > 7 or T-DAR > 27 have a higher risk of IONM loss during pediatric PSF even in the absence of a VCR or three-column osteotomies.

Rate and risk factors for pediatric cervical spine fusion pseudarthrosis: opportunity for improvement.

PURPOSE: Although the pediatric population typically has a high union rate, the cervical spine has a reputation for frequent pseduarthrosis, as high as 38% in some prior series. Our purpose was to examine the rate and risk factors for pseudarthrosis in pediatric cervical spine fusions. METHODS: Retrospective review of all patients with ≥ 2 years follow-up undergoing cervical spinal fusion between January 2004 and December 2019 at a tertiary pediatric hospital. Pseudarthrosis was defined as an absence of radiographic union as assessed by the attending surgeon for which revision surgery was performed. RESULTS: 64 patients (mean age: 8.4 ± 4.7 years) met inclusion criteria. Mean follow-up was 63.3 ± 41.4 months (range: 24-187 months). 28 fusions (44%) included the occiput. 41 patients (64%) had instrumentation, while 23 patients (36%) had uninstrumented fusions. 48 (75%) patients had a halo for a mean of 97.6 ± 49.5 days. The incidence of pseudarthrosis was as follows: overall = 8/64 (12.5%); posterior fusion = 14.8% (8/54); anterior fusions = 0% (0/4); and anteroposterior fusions = 0% (0/6). The rate of pseudarthrosis was over 8 times higher in fusions involving the occiput (occipitocervical fusion: 25.0%; 7/28 vs. cervical alone: 2.8%; 1/36; p = 0.02). Although not statistically significant, the rate of pseudarthrosis was 3 times higher in uninstrumented fusions (21.7%; 5/23) than instrumented fusions (7.3%; 3/41) (p = 0.12). In patients with uninstrumented fusion to the occiput, pseudarthrosis rate was 35.7% (5/14), which was higher compared to those who did not (6.0%; 3/50) (p = 0.01). Incidence of pseudarthrosis was similar in patients who received autograft (13.0%; 7/54) compared to allograft alone (10.0%; 1/10) (p > 0.999). CONCLUSIONS: The pseudarthrosis rate in pediatric cervical spine fusions remained high despite frequent use of halo immobilization and autograft. Patients with uninstrumented occipitocervical fusions are at particularly high risk with more than 1 in 3 developing a pseudarthrosis. STUDY DESIGN: Retrospective, Comparative. LEVEL OF EVIDENCE: III.

Adolescent idiopathic scoliosis patients treated with bracing, surgery, or observation showed no difference in behavioral and emotional function over a 2-year period.

The purpose of this study was to assess if behavior and emotional function, as measured by the Pearson Behavioral Assessment Survey for Children, Second Edition (BASC-2) in patients and parents, changes with differing treatment protocols in patients with adolescent idiopathic scoliosis (AIS). One previous study showed abnormal BASC-2 scores in a substantial number of patients diagnosed with AIS; however, no study has assessed how these scores change over the course of treatment. AIS patients aged 12 to 21 years completed the BASC-2. The 176-item questionnaire was administered to subjects at enrollment, assessing behavioral and emotional problems across 16 subscales of 5 domains: school problems, internalizing problems, inattention/hyperactivity, emotional symptoms index, and personal adjustment. Parents were given an equivalent assessment survey. Surveys were administered again after 2 years. Subject treatment groups (bracing, surgery, and observation) were established at enrollment. Patients were excluded if they did not complete the BASC-2 at both time points. Forty-six patients met the inclusion criteria, with 13 patients in the surgical, 20 in the bracing, and 13 in the observation treatment groups. At enrollment, 26% (12/46) of subjects with AIS had a clinically significant score in 1 or more subscales, and after 2 years 24% (11/46) of subjects reported a clinically significant score in at least 1 subscale (P = .8). There were no significant differences in scores between enrollment and follow-up in any treatment group. Similar to what was reported in a previous study, only 36% (4/11) of patients had clinically significant scores reported by both patient and parent, conversely 64% (7/11) of parents were unaware of their child's clinically significant behavioral and emotional problems. Common patient-reported subscales for clinically significant and at-risk scores at enrollment included anxiety (24%; 11/46), hyperactivity (24%; 11/46), attention problems (17%; 8/46), and self-esteem (17%; 8/46). At 2-year follow-up, the most commonly reported subscales were anxiety (28%; 13/46), somatization (20%; 9/46), and self-esteem (30%; 14/46). Patients with AIS, whether observed, braced or treated surgically, showed no significant change in behavior and emotional distress over the course of their treatment, or compared with each other at 2-year follow-up.

Outcomes of Posterior Spinal Fusion in Pediatric Patients with Down Syndrome.

BACKGROUND: Trisomy 21 or Down syndrome is associated with multiple orthopaedic manifestations. Although cervical instability is the most common spinal condition associated with Down syndrome, the prevalence of scoliosis has been estimated at 4.8% to 8.7%. Very few prior studies have documented the role of spinal fusion in this population, and all have included ≤10 patients. METHODS: An institutional review board-approved multicenter retrospective analysis of patients with Down syndrome treated with spinal fusion between January 2009 and December 2019 was performed by cross-referencing Current Procedural Terminology (CPT) and International Classification of Diseases, Ninth and Tenth Revisions (ICD-9 and ICD-10) codes. Patients were followed for ≥2 years, with a mean follow-up of 3.77 years. Clinical and radiographic outcomes were collected, and complications were documented using the Clavien-Dindo-Sink (CDS) classification. RESULTS: A total of 23 patients were included: 96% had ≥1 medical comorbidities, including 16 (70%) with congenital heart disease, of whom 88% had previous cardiac surgery, and 10 (44%) with thyroid disorders. All 23 patients underwent posterior spinal fusion. The mean estimated blood loss was 617 ± 459 mL, the mean length of the surgical procedure was 290 ± 92.7 minutes, and the mean length of hospital stay was 6.03 ± 2.91 days. The major Cobb angle measured 61.7° ± 17.6°, which corrected to 19.4° ± 14.8° (68.6% correction; p < 0.001), with well-maintained correction at 2 years of 22.0° ± 10.3° (64.3% correction; p = 0.158). Thirteen (57%) of 23 patients had a change in curve of >5°. There were no intraoperative complications; however, 12 patients (52%) sustained postoperative complications (e.g., need for reoperation, implant failure, and pulmonary complications), including 6 patients with CDS type 3 or 4 (e.g., wound dehiscence, late superficial abscess, pleural effusion, pseudarthrosis, and readmission for hypoxia). Four patients (17%) required a revision surgical procedure. One patient (4%) required an unplanned intensive care unit admission. CONCLUSIONS: Although instrumented spinal fusion can effectively correct spinal deformity in these patients, complications are more frequent than in children with adolescent idiopathic scoliosis, with over half of patients sustaining a complication. LEVEL OF EVIDENCE: Prognostic Level III . See Instructions for Authors for a complete description of levels of evidence.

Treatments and Sequelae of Pediatric Pathologic Proximal Femur Fractures Due to Benign Bone Cyst.

BACKGROUND: The proximal femur is a common location for pathologic fractures in children, yet there is little published information regarding this injury. The purpose of this study was to investigate the outcomes of pediatric pathologic proximal femur fractures due to benign bone tumors. METHODS: A retrospective review of patients treated for pathologic proximal femur fractures from 2004 to 2018 was conducted. Inclusion criteria were age below 18 years and pathologic proximal femur fracture secondary to a benign bone tumor. Patients were excluded if they had <1 year of follow-up. Medical charts and serial radiographs were reviewed for fracture classification, underlying pathology, treatment, complications, and time to fracture healing. RESULTS: A total of 14 patients were included. Mean age was 6±3 (3 to 11) years, and mean follow-up was 44±21 (22 to 86) months. Index treatment was spica casting in 9/14 (68%) patients, while 5/14 (32%) were treated with internal fixation. Of the 9 patients initially treated with casting, 22% (2/9) required repeat spica casting at a mean of 0.6 months after index treatment, 67% (6/9) required internal fixation at a mean of 20.3 months after index treatment, and 11% (1/9) did not require revision treatment. Eighty-eight percent (8/9) of patients treated with casting required revision treatment compared with 40% (2/5) of those treated with internal fixation (P=0.05). Nonunion occurred after 1 refracture, malunion with coxa vara occurred in 2 fractures, and the remaining 11/14 (84%) fractures had a union at a mean of 4.9±3.0 months All cases of malunion occurred in patients initially treated nonoperatively. There were 19 distinct complications in 10/14 (71%) patients. The incidence of any revision surgery was 64% (9/14). CONCLUSIONS: In this series, pediatric pathologic proximal femur fractures demonstrated prolonged time to union, high incidence of revision surgery (64%), and substantial complication rate (71%). In children with pathologic proximal femur fractures, treatment with internal fixation is recommended as this series showed a 78% failure rate of initial conservative management. LEVEL OF EVIDENCE: Level IV.