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.

Long-term reoperation rates following spinal fusion for neuromuscular scoliosis in nonambulatory patients with cerebral palsy.

PURPOSE: To describe the incidence of reoperation and factors contributing to surgical revision within a minimum of 10 years after spinal fusion for scoliosis in patients with nonambulatory cerebral palsy (CP). METHODS: We conducted a retrospective review of consecutive nonambulatory patients with CP who underwent primary spinal fusion at a single specialty care center with a minimum of 10 years from their index surgery (surgery dates 2001-2011). Causes of reoperation were classified as implant failure/pseudoarthrosis, surgical site infection (SSI), proximal junctional kyphosis, prominent/symptomatic implants, and implant removal. Reoperation rates with 95% confidence intervals were calculated for each time interval, and an actuarial survival curve was generated. RESULTS: 144 patients met inclusion criteria (mean age = 14.3 ± 2.6 years, 62.5% male); 85.4% had 5 years follow-up data; and 66.0% had 10 years follow-up data. Estimates from the actuarial analysis suggest that 14.9% (95% CI: 10.0-22.0) underwent reoperation by 5 years postsurgery, and 21.7% (95% CI: 15.4-30.1) underwent reoperation by 10 years postsurgery. The most common causes for reoperation were implant failure/pseudoarthrosis, SSI, and prominent/symptomatic implants. CONCLUSIONS: To our knowledge, this study is the largest long-term follow-up of nonambulatory patients with CP and neuromuscular scoliosis who underwent spinal fusion. Approximately 22% of these patients required reoperation 10 years after their index surgery, primarily due to implant failure/pseudoarthrosis, SSI, and prominent/symptomatic implants. Complications and reoperations continued throughout the 10 years period after index surgery, reinforcing the need for long-term follow-up as these patients transition into adulthood. LEVEL OF EVIDENCE: III.

The effects of gastrojejunostomy tube placement on pulmonary and gastrointestinal complications following spinal fusion for neuromuscular scoliosis.

To evaluate whether preoperative conversion from a gastrostomy tube (G-tube) to a gastrojejunostomy tube (GJ-tube) decreases short-term postoperative aspiration pneumonia and gastrointestinal complications in children with neuromuscular scoliosis. We conducted a retrospective chart review from January 2006 to October 2021 of pediatric patients who had neuromuscular scoliosis and were fed with a G-tube before spinal fusion. Eligible patients were divided into two groups based on whether they were converted to a GJ-tube preoperatively. Preoperative characteristics and 30-day postoperative outcomes were compared between groups using Chi-square tests. Of 261 eligible patients, 205 were converted to a GJ-tube, while 56 underwent spinal fusion with a G-tube. Common complications following G-tube to GJ-tube conversion were feeding intolerance (25.2%), GJ-tube malfunction (17.7%), and at least one episode of vomiting (17.4%). Within 30 days of discharge, 12.5% of GJ-tube patients and 11.5% of G-tube patients experienced aspiration pneumonia (P = 0.85). The GJ-tube group received postoperative tube feeds 7 hours earlier than the G-tube group on average (51.6 h vs. 44.5 h, P = 0.02). Within 30 days of discharge, one (0.5%) patient from the GJ-tube group died of gastrointestinal complications unrelated to conversion and two (3.6%) patients in the G-tube group died from aspiration pneumonia (P = 0.12). Results suggest that there were no appreciable differences in outcomes between patients converted to a GJ-tube preoperatively compared to those who continued to use a G-tube. However, preoperative characteristics indicate that a higher number of complex patients were converted to a GJ-tube, indicating potential selection bias in this retrospective sample. Level of evidence: Level III.

An in vivo analysis of implanted programmable device interference during magnetically controlled growing rod lengthenings: a story of 129 lengthenings.

PURPOSE: Early-onset scoliosis (EOS) is often treated with magnetically controlled growing rods (MCGR) which can be lengthened with a magnetic external remote control (ERC). Many individuals with EOS have concomitant medical conditions which are managed with other implanted programmable devices (IPD). Some providers are concerned that the magnetic field generated during MCGR lengthening may interfere with other IPD, such as ventriculoperitoneal shunts (VPS), intrathecal baclofen pumps (ITBP), vagal nerve stimulators (VNS), and cochlear implants (CI). The aim of this study was to evaluate the safety of MCGR lengthenings in patients with EOS and other IPD. METHODS: This single-center, single-surgeon case series followed 12 patients with 13 IPD as they underwent treatment with MCGR. Post MCGR lengthening, monitoring of patient symptoms, and interrogation of IPD were conducted to evaluate for magnetic interference. RESULTS: After 129 MCGR lengthenings, post-lengthening VPS interrogation found 2 instances of potential interference in settings (both in Medtronic Strata shunts); however, no pre-lengthening interrogation was completed to confirm if these changes occurred prior to or during the lengthening procedure. ITBP interrogation found no changes, and there were no patient-reported adverse effects related to VNS or CI function. CONCLUSION: It is safe and effective to utilize MCGR in patients with IPD. However, the possibility of magnetic interference must be considered, particularly in individuals with VPS. We recommend approaching with the ERC from a caudal direction to minimize potential interference and all patients be monitored during treatment. If possible, IPD settings should be assessed pre-lengthening, confirmed afterwards and readjusted if necessary. LEVEL OF EVIDENCE: Level IV.

Pitfalls and management of corrective spinal surgery in trisomy 9 mosaicism: a report of three cases.

PURPOSE: The aim of this case series is to present the outcomes of surgical correction of hyperkyphosis, and subsequent management of complications for three patients with a rare chromosomopathy, Trisomy 9 Mosaicism (T9M). METHODS: Case series with 2 year outcomes following index surgery. RESULTS: Case 1 presented at 9 years of age with 103° of kyphosis (T3-T10), which progressed to 118° despite bracing. Case 2 presented at 7 years of age with 113° of kyphosis (T3-T11). Case 3 presented at 4 years of age with 103° of kyphosis (T1-T11). Cases 1 and 2 underwent T2-L2 posterior instrumented spinal fusion (PISF). Upon follow up, radiographs for Cases 1 and 2 revealed severe, symptomatic proximal junctional kyphosis (PJK) of 71° and 50°, respectively, requiring surgical revision proximally to the C4 level. Case 3 underwent placement of magnetically controlled growing rods given young age and growth potential. Surgical levels for Case 3 were extended from C4-pelvis in an attempt to prevent development of symptomatic PJK. Most recent radiographs for Case 3 taken 21 months postoperatively demonstrate a stable 50° of kyphosis. PJK above C4 was noted but is stable and asymptomatic. CONCLUSION: T9M often presents with progressive hyperkyphosis. Despite instrumentation above the upper end vertebra (UEV), PJK may be a common complication in this small patient population. This novel report on spine deformity correction in the T9M population may provide preliminary guidance for the treatment of hyperkyphosis in patients with T9M and help surgeons avoid common pitfalls. LEVEL OF EVIDENCE: IV.

Current Evidence Regarding the Treatment of Pediatric Lumbar Spondylolisthesis: A Report From the Scoliosis Research Society Evidence Based Medicine Committee.

STUDY DESIGN: Structured literature review. OBJECTIVES: The Scoliosis Research Society requested an assessment of the current state of peer-reviewed evidence regarding pediatric lumbar spondylolisthesis to identify what is known and what research remains essential to further understanding. SUMMARY OF BACKGROUND DATA: Pediatric lumbar spondylolisthesis is common, yet no formal synthesis of the published literature regarding treatment has been previously performed. METHODS: A comprehensive literature search was performed. From 6600 initial citations with abstract, 663 articles underwent full-text review. The best available evidence regarding surgical and medical/interventional treatment was provided by 51 studies. None of the studies were graded Level I or II evidence. Eighteen of the studies were Level III, representing the current best available evidence. Thirty-three of the studies were Level IV. RESULTS: Although studies suggest a benign course for "low grade" (<50% slip) isthmic spondylolisthesis, extensive literature suggests that a substantial number of patients present for treatment with pain and activity limitations. Pain resolution and return to activity is common with both medical/interventional and operative treatment. The role of medical/interventional bracing is not well established. Uninstrumented posterolateral fusion has been reported to produce good clinical results, but concerns regarding nonunion exist. Risk of slip progression is a specific concern in the "high grade" or dysplastic type. Although medical/interventional observation has been reported to be reasonable in a small series of asymptomatic high-grade slip patients, surgical treatment is commonly recommended to prevent progression. There is Level III evidence that instrumentation and reduction lowers the risk of nonunion, and that circumferential fusion is superior to posterior-only or anterior-only fusion. There is Level III evidence that patients with a higher slip angle are more likely to fail medical/interventional treatment of high-grade spondylolisthesis. CONCLUSIONS: The current "best available" evidence to guide the treatment of pediatric spondylolisthesis is presented. LEVEL OF EVIDENCE: Level III; review of Level III studies.

Spinal Deformity Associated with Chiari Malformation.

Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not necessary and scoliosis has been described in the absence of a syrinx. Decompression of the hindbrain is often recommended. In young patients (<10 years old) and/or those with small coronal Cobb measurements (<40°), decompression of the hindbrain may lead to resolution of the spinal deformity. Spinal fusion is reserved for those curves that progress to deformities greater than 50°. Further research is needed to understand the underlying pathophysiology to improve prognostication and treatment of this patient population.

Clinical outcome of deep wound infection after instrumented posterior spinal fusion: a matched cohort analysis.

STUDY DESIGN: Retrospective case control study. OBJECTIVE: Determine the impact of infection on clinical outcome in patients undergoing posterior spinal fusion surgery. SUMMARY OF BACKGROUND DATA: The outcome of patients treated for infection after spinal surgery is not well established because of variability in cohort identification, definition of infection, outcomes instrument, use of a control group, and/or sample size. METHODS: Thirty-two patients were included. Sixteen patients ("infection group") met inclusion criteria of deep wound infection after spinal fusion with posterior segmental instrumentation (including combined approach). A 1:1 matched cohort ("control group") was created based on primary or revision status, length of fusion, diagnosis, and age. Postoperative patient outcomes were evaluated using the physical components of SF-36 v2.0 with minimum 2-year follow-up. RESULTS: No significant difference in the Physical Function, Role Physical, Bodily Pain, and General Health domains was detected between the infection group and control group. Mean follow-up was 62 months. Mean Physical Component Summary was 41.4 in the infection group and 44.3 in the control group (P = 0.6). Infection occurred early in 12 patients and late in 4 patients. Most common organisms isolated were Staphylococcus epidermidis, Enterococcus sp., and Staphylococcus aureus. Multiple debridements were significantly associated with polymicrobial infections and later pseudarthrosis requiring reoperation. CONCLUSION: An aggressive approach to deep wound infection emphasizing early irrigation and debridement allowed preservation of instrumentation and successful fusion in most cases. At the conclusion of treatment, patients can expect a medium-term clinical outcome similar to patients in whom this complication did not occur.

Analysis of paired primary lung and lymph node tumor cells: a model of metastatic potential by multiple genetic programs.

BACKGROUND: The current paradigm of metastasis proposes that rare cells within primary tumors acquire metastatic capability via sequential mutations, suggesting that metastases are genetically dissimilar from their primary tumors. We tested this hypothesis by examining the molecular differences, if any, between primary tumor cells and matched lymph node metastatic cells in human non-small-cell lung carcinoma specimens. METHODS: We performed transcriptional profiling studies on malignant cells from 11 pairs of stage III tumors and their tumor-positive lymph nodes using multiple, complementary analytic techniques. To confirm the overall validity of microarray data, we used real-time polymerase chain reaction. RESULTS: The molecular signature of nodal metastasis was a composite of two paradoxical, but not mutually exclusive, expression patterns: metastatic cells are: (1) different from their primary tumor cells based on a few genes and (2) genetically similar, overall, to their primary tumor cells. Consequently, we found a 27-gene subset sufficient to differentiate nodal metastatic cells from primary tumor cells. CONCLUSIONS: Thus, we concluded that a more accurate model of metastatic potential is based on a global primary tumor expression pattern along with the appearance of distinct metastatic variants. The 27-gene signature differentiating primary tumors from their metastatic cells may define non-small-cell lung carcinoma nodal metastatic potential.

Selective activation of insulin receptor substrate-1 and -2 in pleural mesothelioma cells: association with distinct malignant phenotypes.

Molecular mechanisms active in transforming human pleural cells remain incompletely understood. Our previous microarray analysis of malignant pleural mesothelioma revealed alterations in components of the insulin-like growth factor (IGF) system, implicating this signaling axis in tumorigenesis. Therefore, in this current study, we characterized the molecular phenotype and investigated the key signaling pathways of the IGF system in malignant pleural mesothelioma specimens. For the major IGF components, we assessed mRNA abundance and total protein levels. We measured IGF-I ligand-dependent activation of signaling pathways downstream of the type I IGF receptor in a subset of malignant pleural mesothelioma cell lines and determined the corresponding biological consequences. At the transcriptional level, we observed consistent changes in IGF components that may contribute to a malignant phenotype. IGF-I stimulation of cells resulted in enhanced activation of type I IGF receptor and IRS adaptor proteins. Differential activation of IRS-1 signaling was associated with cell growth, whereas IRS-2 signaling was associated with cell motility. Thus, these data suggest that multiple mechanisms likely contribute to malignant pleural mesothelioma tumorigenesis. Therefore, IGF system components represent novel malignant pleural mesothelioma therapeutic targets for investigation.