Rib-on-Pelvis Deformity and Reported Pain in Neuromuscular Early-Onset Scoliosis.

BACKGROUND: Neuromuscular early-onset scoliosis (N-EOS) often presents with a long sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the pelvis, termed rib-on-pelvis deformity (ROP). The goal of this study is to evaluate whether ROP is associated with reported pain and other health-related quality of life (HRQOL) measures. We hypothesize that ROP is associated with increased pain and negative HRQOL. METHODS: A multicenter international registry was queried for all nonambulatory patients with N-EOS from 2012 to 2022. Both surgical and nonsurgical patients were included. ROP was classified as a binary radiographic assessment of preoperative (surgical patients) and most recent follow-up (nonsurgical patients) upright radiographs. Reported pain and other HRQOL measures were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). Patients with nonupright radiographs or EOSQ-24 questionnaires and corresponding radiographs >4 months apart were excluded. RESULTS: Totally, 225 patients (8.4±3.1 y, 55% female) were included. The median major curve was 63.3 (IQR: 40.6 to 81.2) degrees and median pelvic obliquity was 15.5 degrees (IQR: 8.8 to 26.4). Eighty-three patients (37%) had ROP. ROP was associated with both frequency (P<0.001) and severity (P<0.001) of pain. ROP was associated with worse general health (P=0.01), increased difficulty with vocalization (P=0.02), increased frequency of shortness of breath (P=0.002), and increased difficulty sitting upright (P=0.04). Regarding overall EOSQ-24 domains, ROP was associated with worse general health, pain/discomfort, pulmonary function, and physical function (P<0.01). In a subanalysis of 76 patients who underwent surgical intervention with at least 2 years of follow-up, patients with preoperative ROP experienced significantly greater improvements in both frequency (P=0.004) and severity (P=0.001) of pain than the patients without preoperative ROP at 2 years postoperatively. CONCLUSIONS: The overall incidence of ROP in N-EOS is about 37%. ROP is associated with greater pain and worse HRQOL through the EOSQ-24 questionnaire. Furthermore, these patients experienced a greater reduction in pain after surgery. Clinicians and parents must be aware that ROP is possibly a pain generator, but responds positively to surgical intervention. LEVEL OF EVIDENCE: Level III.

Proximal foundation anchor variations and their correlation with unplanned return to the operating room (UPROR) in children with EOS treated with magnetically controlled growing rods (MCGR).

INTRODUCTION: The evolution of MCGR technique has led to modifications in the configuration of the proximal construct to decrease the incidence of implant-related complications (IRC) and revision surgeries. However, there is no data characterizing the performance of the most used configurations reducing the risk of complications. METHODS: 487 patients were identified from an international multicenter EOS database. INCLUSION CRITERIA: EOS patients, primary dual MCGR, complete radiographs, and minimum of 2-year follow-up. 76 patients had incomplete X-rays, 5 had apical fusions, and 18 had inconclusive complications, leaving 388 patients for review. A digital spine template was created to document UIV; number of levels; number, type, and location of anchors; as well as implant configuration. First available postoperative and latest follow-up radiographs were reviewed by two senior surgeons and two spine fellows. UPROR due to IRC was defined as any change in proximal anchors between the postoperative and final follow-up radiographs. RESULTS: The most common proximal construct configuration: UIV at T2 (50.0%) with 17.5% UPROR, followed by T3 (34.0%) with 12.1% UPROR; number of levels was three (57.1%) with 16.8% UPROR and two (26.0%) with 17.0% UPROR; number of proximal anchors was six (49.9%) with 14.1% UPROR and four (27.0%) with 18.3% UPROR. The most common anchors were all screws (42.0%) with 9.9% UPROR, and all hooks (26.4%) with 31.4% UPROR (P < 0.001). The construct with the lowest rate of UPROR was a UIV at T2, with six anchors (all screws) across three levels (42 cases), with 0% UPROR. Other construct combinations that yielded 0% UPROR rates were UIV of T3, six anchors (all screws) across three levels (25 cases), and a UIV of T3 with six anchors (screws and hooks) across three3 levels (9 cases). CONCLUSION: Proximal anchor configuration impacts the incidence of UPROR due to IRC in MCGR. UIV at T2 and T3 compared to T4, and the use of all screws or combination of screws and hooks compared to all hooks were associated with a lower UPROR rate. The most common construct configuration was T2 UIV, three levels, six anchors, and all screws. The use of a combination of six anchors (screws or screws and hooks) across three levels with a UIV at T2 or T3 was associated with a lower UPROR rate. Additional research is needed to further evaluate the variables contributing to configuration selection and their association with IRC.

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.

How Many Imaging Studies Do Patients With Neuromuscular Early Onset Scoliosis (EOS) Receive?

BACKGROUND: Children with neuromuscular early onset scoliosis (EOS) receive numerous radiographic studies both from orthopaedic and other specialties. Ionizing radiation doses delivered by computed tomography (CT) are reportedly 100 times higher than conventional radiography. The purpose of this study was to evaluate the number of radiographic studies ordered for neuromuscular EOS patients during their care. METHODS: Retrospective review at a tertiary children's hospital from January 2010 to June 2021 included all patients with neuromuscular EOS followed by an orthopaedic specialist for a minimum of 3 years. Patients were excluded if the majority of their nonorthopaedic care was provided by outside institutions. RESULTS: Eighteen patients met inclusion criteria with mean follow up of 6.4±2.3 years. A total of 1312 plain radiographs and 35 CT scans were performed. Of the plain radiographs, 34.7% were ordered by orthopaedic providers and 65.3% (857/1312) were ordered by other providers. Of the CT scans, 4 were ordered by orthopaedic providers, while 88.5% (21/35) were ordered by other providers. An average of 74.7 (range: 29 to 124) radiographs and 1.9 (range: 0 to 9) CT scans ordered over the course of each patient's treatment for an average of 13.0±6.0 radiographs and 0.3 CT scans per year. CONCLUSIONS: With an average of 75 radiographs and 1.9 CT scans performed per patient, consideration for steps to limit exposure to ionizing radiation should be made a particularly high priority in this unique subset of patients. This requires interdisciplinary coordination as 65% of the radiographs and over 80% of the CT scans were ordered by nonorthopaedic providers. LEVEL OF EVIDENCE: Level 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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Paediatric syndromic scoliosis: proceedings of the half-day course at the 57th annual meeting of the Scoliosis Research Society.

There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality. This review explores the features of these syndromes underpinning the aspects of surgical and medical management. This review represents the proceedings of the Paediatric Half-Day Course at the 57th Annual Meeting of the Scoliosis Research Society.

Increasing Cervical Kyphosis Correlates With Cervical Degenerative Disk Disease in Patients With Adolescent Idiopathic Scoliosis.

STUDY DESIGN: Retrospective review. OBJECTIVE: Our goal was to investigate the incidence of cervical degenerative disk disease (DDD) in patients with adolescent idiopathic scoliosis (AIS), before surgical intervention. SUMMARY OF BACKGROUND DATA: AIS is often associated with thoracic hypokyphosis and compensatory cervical kyphosis. In adults, cervical kyphosis is associated with DDD. Although cervical kyphosis has been reported in up to 60% AIS patients, the association with cervical DDD has not been reported. MATERIALS AND METHODS: A retrospective review was conducted from January 2014 to December 2019 of all consecutive AIS patients. Inclusion criteria were AIS patients over 10 years of age with cervical magnetic resonance imaging and anterior-posterior and lateral spine radiographs within 1 year of each other. Magnetic resonance imaging were reviewed for evidence of cervical DDD. Severity of cervical changes were graded using the Pfirrmann classification and by a quantitative measure of disk degeneration, the magnetic resonance signal intensity ratio. RESULTS: Eighty consecutive patients were included (mean age: 14.1 years, SD=2.5 years). Increasing cervical kyphosis was significantly correlated to decreasing thoracic kyphosis ( r =0.49, P <0.01) and increasing major curve magnitude ( r =0.22, P =0.04). Forty-five patients (56%) had the presence of DDD (grades 2-4) with a mean cervical kyphosis of 11.1° (SD=9.5°, P <0.01). More cervical kyphosis was associated with more severe cervical DDD as graded by Pfirrmann classification level ( P <0.01). Increasing cervical kyphosis was also positively associated with increasing magnetic resonance signal intensity ratio ( P <0.01). Nine patients had ventral cord effacement secondary to DDD with a mean cervical kyphosis of 22.8° (SD=8.6°) compared with 2.6° (SD=11.2°) in those who did not ( P <0.01). CONCLUSIONS: Cervical kyphosis was significantly associated with increasing severity of cervical DDD in patients with AIS. Patients with evidence of ventral cord effacement had the largest degree of cervical kyphosis with a mean of 22.8±8.6°. This is the first study to evaluate the association between cervical kyphosis in AIS with cervical DDD.

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.

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.

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.