BACKGROUND AND OBJECTIVES: The optimal management strategy for pediatric patients with symptomatic moyamoya disease (MMD) is not well established. This systematic review and meta-analysis compares surgical vs conservative management and direct/combined bypass (DB/CB) vs indirect bypass (IB) for pediatric patients with symptomatic MMD. METHODS: MEDLINE and PubMed were searched from inception to March 17, 2024. For analysis of surgical vs conservative treatment, the primary and secondary outcomes were follow-up ischemic stroke and intracranial hemorrhagic events, respectively. For analysis of DB/CB vs IB, the primary outcome was follow-up ischemic stroke, and secondary outcomes included follow-up transient ischemic attack, new or worsened seizures, symptomatic improvement, modified Rankin Scale score ≤2, and Matsushima grade A at the last follow-up. RESULTS: Twenty-two included studies yielded 1091 patients, with a median follow-up duration of 35.7 months. Regarding surgical vs conservative management, 428 patients were analyzed. Surgical treatment was associated with lower odds of ischemic stroke (odds ratios [OR] = 0.33 [95% CI, 0.11-0.97], P = .04), and intracranial hemorrhagic events tended to be lower with surgery (OR = 0.25 [0.06-1.03], P = .05). Regarding DB/CB techniques vs IB, 875 patients were analyzed. The groups had similar rates of ischemic stroke (OR = 0.79 [0.31-1.97], P = .61), transient ischemic attack (OR = 1.27[0.46-3.55], P = .64), new or worsened seizures (OR = 1.05[0.3-3.65], P = .93), symptomatic improvement (OR = 2.45[0.71-8.45], P = .16), and follow-up modified Rankin Scale ≤2 (OR = 1.21 [0.16-8.85], P = .85). CB was associated with higher Matsushima grade A relative to IB (OR = 3.44 [1.32-9.97], P = .01). CONCLUSION: Surgical revascularization yielded more favorable clinical outcomes than conservative management in this meta-analysis. Clinical outcomes were similar between DB/CB vs IB techniques. Surgical flow augmentation, either by DB/CB or IB, seems to benefit pediatric patients with symptomatic MMD.

OBJECTIVE The aim of this study was to delineate the clinical and socioeconomic variables associated with shunt revision in pediatric patients presenting to the emergency department (ED) with concerns of ventricular shunt malfunction. METHODS A retrospective analysis of pediatric ED consultations for shunt malfunction over a 1-year period was conducted, examining clinical symptoms, radiographic findings, and socioeconomic variables. Sensitivities, specificities, and positive and negative predictive values were calculated for each presenting symptom collected. Logistic regression models were used to estimate the odds ratios for shunt revision based on these variables, and multivariate analyses were used to adjust for potential confounders. RESULTS Of the 271 ED visits from 137 patients, 19.2% resulted in shunt revision. Increased ventricle size on imaging (OR 11.38, p < 0.001), shunt site swelling (OR 9.04, p = 0.01), bradycardia (OR 7.08, p < 0.001), and lethargy (OR 5.77, p < 0.001) were significantly associated with shunt revision. Seizure-like activity was inversely related to revision needs (OR 0.24, p < 0.001). Patients with private or self-pay insurance were more likely to undergo revision compared with those with public insurance (p = 0.028). Multivariate analysis further confirmed the significant associations of increased ventricle size, lethargy, and bradycardia with shunt revision, while also revealing that seizure-like activity inversely affected the likelihood of revision. Patients with severe cognitive and language disabilities were more likely to be admitted to the hospital from the ED but were not more likely to undergo revision. CONCLUSIONS Clinical signs such as increased ventricle size, shunt site swelling, bradycardia, and lethargy may be strong predictors of the need for shunt revision in pediatric patients presenting to the ED with concerns of shunt malfunction. Socioeconomic factors play a less clear role in predicting shunt revision and admission from the ED; however, the nature of their influence is unclear. These findings can help inform clinical decision-making and optimize resource utilization in the ED.

Background Tension in the spinal cord is a trademark of tethered cord syndrome. Unfortunately, existing tests cannot quantify tension across the bulk of the cord, making the diagnostic evaluation of stretch ambiguous. A potential non-destructive metric for spinal cord tension is ultrasound-derived shear wave velocity (SWV). The velocity is sensitive to tissue elasticity and boundary conditions including strain. We use the term Ultrasound Tensography to describe the acoustic evaluation of tension with SWV. Methods Our solution Tethered cord Assessment with Ultrasound Tensography (TAUT) was utilized in three sub-studies: finite element simulations, a cadaveric benchtop validation, and a neurosurgical case series. The simulation computed SWV for given tensile forces. The cadaveric model with induced tension validated the SWV-tension relationship. Lastly, SWV was measured intraoperatively in patients diagnosed with tethered cords who underwent treatment (spinal column shortening). The surgery alleviates tension by decreasing the vertebral column length. Results Here we observe a strong linear relationship between tension and squared SWV across the preclinical sub-studies. Higher tension induces faster shear waves in the simulation ( R 2 = 0.984) and cadaveric ( R 2 = 0.951) models. The SWV decreases in all neurosurgical procedures ( p < 0.001). Moreover, TAUT has a c-statistic of 0.962 (0.92-1.00), detecting all tethered cords. Conclusions This study presents a physical, clinical metric of spinal cord tension. Strong agreement among computational, cadaveric, and clinical studies demonstrates the utility of ultrasound-induced SWV for quantitative intraoperative feedback. This technology is positioned to enhance tethered cord diagnosis, treatment, and postoperative monitoring as it differentiates stretched from healthy cords.

Myelomeningocele (MMC) is a severe form of spina bifida characterized by spinal cord extrusion into a cerebrospinal fluid (CSF) filled sac which may lead to lifelong disability. Repair of these lesions have classically occurred shortly after birth, but more recently, prenatal myelomeningocele repair techniques have been elucidated. This study aimed to investigate the outcome of surgery, particularly with subsequent spinal cord tethering, in patients with prenatal myelomeningocele closure and those with postnatal repair surgery. In this retrospective study which assessed patients from April 2002 to April 2020, the data of 18 total patients with MMC were reviewed. Nine patients from each group were included, closed prenatally or postnatally, respectively. Demographic information including age and sex, birth week, infant comorbidities, presence of dermoid or lipoma, cutaneous stigmata, total number and timing of detethering procedures, presence of Chiari malformation, and need for VPS was obtained. Eighteen patients including nine infants closed prenatally and nine infants with myelomeningoceles closed postnatally were reviewed. The mean age was 4 ± 3 years and 6.22 ± 2.4 in prenatal and postnatal retrospectively. There was a significant relationship between the number of detethering procedures (p-value = 0.03) and the need for a ventriculoperitoneal shunt (VPS) (p-value = 0.01) between the groups, with the prenatal closure group having lower rates of each. There was no significant difference between the groups in regard to the mean age at the detethering procedure (p = 0.4), sex (p = 0.09), birth week (p = 0.8), comorbidities (p = 0.8), presence of intraspinal dermoid or lipoma (p = 0.09), presence of cutaneous stigma (p = 0.08), Chiari (p = 0.6), fatty filum (p = 0.08), syrinx (p = 0.4), bone anomaly (p = 0.4), and spina bifida neurological scale (p = 0.66). There was a significant relationship between the two groups in terms of the number of detethering procedures, and the need for VPS. Our data represents a possible difference in the need for detethering surgeries and ventriculoperitoneal shunt placements between patients with prenatal and postnatal myelomeningocele closures.

OBJECTIVE:Gliosarcoma (GSM) is a variant of glioblastoma, 1 of the most common and aggressive primary brain tumors in adults. Our study seeks to analyze a large cohort of patients with GSM in the National Cancer Database (NCDB) to elucidate clinical predictors of overall survival (OS). METHODS:Data was collected on patients diagnosed with histologically-confirmed GSM using the NCDB (2004-2016). OS was determined via univariate Kaplan-Meier analysis. Bivariate and multivariate Cox proportional-hazards analyses were also utilized. RESULTS:Our cohort of 1015 patients had a median age at diagnosis of 61 years. Six hundred thirty-one (62.2%) were male, 896 (89.0%) were Caucasian, and 698 (68.8%) lacked any comorbidities. Median OS was 11.5 months. Regarding treatment, 264 (26.5%) patients underwent surgery (S) only (OS = 5.19 months), 61 (6.1%) underwent surgery and radiotherapy (S + RT) (OS = 6.87 months), 20 (2.0%) underwent surgery and chemotherapy (S + CT); (OS = 15.51 months), and 653 (65.4%) underwent S + CT + RT (triple) combination therapy (OS = 13.8 months). Notably, on bivariate analysis, S + CT (Hazard ratio [HR] = 0.59, P-value = 0.04) and triple therapy (HR = 0.57, P < 0.01) were associated with increased OS. S + RT was not significantly associated with OS. Similarly, on multivariate Cox proportional-hazards analyses, gross total resection (HR = 0.76, P = 0.02), S + CT (HR = 0.46, P < 0.01), and triple therapy (HR = 0.52, P < 0.01) predicted significantly increased OS. Furthermore, age >60 years old (HR = 1.03, P < 0.01) and the presence of comorbidities (HR = 1.43, P < 0.01) predicted significantly decreased OS. CONCLUSIONS:Despite maximal multimodal treatment, GSMs have poor median OS. NCDB data suggest age, comorbidities, extent of resection, and adjuvant treatment each minimally delays poor outcomes.

Hypothalamic hamartomas (HHs) are rare lesions typically found in pediatric patients with gelastic seizures, precocious puberty, and rage attacks.(1) Treatment is surgical, ranging from less invasive approaches such as laser interstitial thermal therapy to open craniotomies for resection.(2-4) Orbitozygomatic craniotomies provide a skull base approach with excellent visualization of the inferior hypothalamic region and access to the third ventricle through the lamina terminalis with excellent postoperative cosmetic results.(4-6) The use of this approach for HH has been reported; however, details regarding the technical nuances of resection are sparse. Advances in intraoperative imaging using a surgical exoscope have also allowed for better visualization of the skull base anatomy and more ergonomic operations for the surgeon. Here, we report a case and provide a high-quality 2-dimensional exoscopic video detailing the use of an orbitozygomatic craniotomy for the resection of a giant HH, which had failed prior laser interstitial thermal therapy in a pediatric patient with persistent gelastic seizures and precocious puberty. Postoperative MRI demonstrated gross total resection of the lesion, and the patient was discharged at his neurological baseline. The patient remained seizure-free at the 6-month follow-up visit. The patient's family consented to participate in the surgery and the surgical video. The patient's family consented to the publication of his image.

Abstract Background Tension in the spinal cord is a trademark of tethered cord syndrome. Unfortunately, existing tests cannot quantify tension across the bulk of the cord, making the diagnostic evaluation of stretch ambiguous. A potential non-destructive metric for spinal cord tension is ultrasound-derived shear wave velocity (SWV). The velocity is sensitive to tissue elasticity and boundary conditions including strain. We use the term Ultrasound Tensography to describe the acoustic evaluation of tension with SWV. Methods Our solution “Tethered cord Assessment with Ultrasound Tensography (TAUT)” was utilized in three sub-studies: finite element simulations, a cadaveric benchtop validation, and a neurosurgical case series. The simulation computed SWV for given tensile forces. The induced tension cadaveric model validated the SWV-tension relationship. Lastly, SWV was measured intraoperatively in patients diagnosed with tethered cord who underwent surgical treatment (spinal column shortening). The surgery alleviates tension by decreasing the vertebral column length. Results Here we observe a strong linear relationship between tension and squared SWV across the preclinical sub-studies. Higher tension induces faster shear waves in the simulation (R2 = 0.984) and cadaveric (R2 = 0.951) models. The SWV decreases in all neurosurgical procedures (p<0.001). Moreover, TAUT has a c-statistic of 0.962 (0.92-1.00), detecting all tethered cords. Conclusions This study presents the first clinical metric of spinal cord tension. Strong agreement among computational, cadaveric, and clinical studies demonstrates the utility of ultrasound-induced SWV for quantitative intraoperative feedback. This technology is positioned to enhance tethered cord diagnosis, treatment, and post-operative monitoring as it differentiates stretched from healthy cords.

Central nervous system (CNS) tumors are the leading cause of cancer death in pediatric patients. Though these tumors typically require invasive surgical procedures to diagnose, cerebrospinal fluid (CSF) liquid biopsy presents a potential method for rapid and noninvasive detection of markers of CNS malignancy. To characterize molecular biomarkers that can be used in the diagnosis, prognosis, and monitoring of pediatric cancer patients, a literature review was conducted in accordance with PRISMA guidelines. PubMed and EMBASE were searched for the terms biomarkers, liquid biopsy, cerebrospinal fluid, pediatric central nervous system tumor, and their synonyms. Studies including pediatric patients with CSF sampling for tumor evaluation were included. Studies were excluded if they did not have full text or if they were case studies, methodology reports, in languages other than English, or animal studies. Our search revealed 163 articles of which 42 were included. Proteomic, genomic, and small molecule markers associated with CNS tumors were identified for further analysis and development of detection tools.

Spinal cord injury (SCI) is a devastating condition that affects about 17,000 individuals every year in the United States, with approximately 294,000 people living with the ramifications of the initial injury. After the initial primary injury, SCI has a secondary phase during which the spinal cord sustains further injury due to ischemia, excitotoxicity, immune-mediated damage, mitochondrial dysfunction, apoptosis, and oxidative stress. The multifaceted injury progression process requires a sophisticated injury-monitoring technique for an accurate assessment of SCI patients. In this narrative review, we discuss SCI monitoring modalities, including pressure probes and catheters, micro dialysis, electrophysiologic measures, biomarkers, and imaging studies. The optimal next-generation injury monitoring setup should include multiple modalities and should integrate the data to produce a final simplified assessment of the injury and determine markers of intervention to improve patient outcomes.

INTRODUCTION:Post-traumatic syringomyelia is an uncommon complication after traumatic spinal cord injury. This case study details our decision-making and surgical approach for a patient with symptomatic post-traumatic syringomyelia after sustaining a gunshot wound.CASE PRESENTATION:A 24-year-old man with past medical history of distant American Spinal Injury Association Impairment Grade B spinal cord injury due to ballistic injury developed delayed post-traumatic syringomyelia, resulting in unilateral sensory loss and left upper extremity weakness. CT and MR imaging revealed a syrinx spanning his cervical and thoracic spine causing significant spinal cord compression. To relieve achieve decompression and restore CSF flow dynamics, we performed a bony extradural decompression, bullet fragment extraction, spinal cord untethering, and midline myelotomy. Postoperatively, the patient demonstrated clinical and radiographical improvement.DISCUSSION:Post-traumatic syringomyelia is potentially morbid sequalae of spinal cord injuries. Suspicion for post-traumatic syringomyelia should be maintained in patients with delayed, progressive neurologic deficits. In this setting, surgical intervention may require extradural and intradural procedures to mitigate neural compression along the dilated central canal by the syrinx.

IntroductionRecently, there has been a paradigm shift in the management of hypothalamic hamartoma (HH) from traditional microsurgical techniques to less invasive alternatives such as stereotactic radiosurgery and laser interstitial thermal therapy. However, large and extensive HH may fail to respond to minimally invasive therapies, ultimately necessitating microsurgery.MethodsAll patients who underwent microsurgical resection of a complex HH by the 2 senior authors (D.J.L., H.L.R.) in 2011-2017 were included. Charts were retrospectively reviewed and demographic, clinical, imaging, and outcome data were recorded.Results8 patients, 7 children and 1 adult, with a mean age of 7 years (10 months-27 years), were included. Of those, 2 had failed previous treatments. All 7 children presented with pharmacoresistant gelastic seizures and cognitive dysfunction, 6 exhibited central precocious puberty, and 3 had behavioral problems. Other seizure types affected 6/8 patients. Mean lesion size was 21.6 mm (14-31), all with interpeduncular extension and 5 with intraventricular extension (Delalande type I: 3, type III: 4, type IV: 1). A frontotemporal orbitozygomatic (FTOZ) approach with optic nerve decompression was used in all patients, supplemented by another approach in 3 (endoscopic transventricular: 3, transcallosal: 1). Gross total resection was achieved in 6 patients and subtotal resection in 2. Transient complications occurred in 3 patients (37.5%): self-limited sodium imbalance (n=3), subdural hygroma (n=2). Permanent complications occurred in 2 patients (25%): perforator infarct (n=1), short-term memory loss (n=1). All patients experienced seizure resolution postoperatively with preserved hypothalamic-pituitary axis function. After a mean follow-up of 41 months (2-66), 7 patients remain seizure-free, while 1 has rare recurrent seizures. Cognitive and behavioral symptoms improved significantly in all patients.ConclusionFor large HH with interpeduncular extension, microsurgery via the FTOZ approach is a safe and highly effective treatment modality.

A middle-aged woman who underwent a recent lumbar laminectomy and discectomy presents with leakage from her incision and positional headaches. Neurosurgery is consulted for management.

A middle-aged man with a history of metastatic prostate cancer presents with sudden onset of bilateral lower extremity weakness and hemodynamic instability. Imaging demonstrates no compressive findings but T2 hyperintensity from T1-T7 within the spinal cord, and the team is consulted for management. The differential for intrinsic, intramedullary lesions of the spinal cord are reviewed.

Study Design: Retrospective cohort study. Objective: The aim was to investigate the accuracy of pedicle screw placement by freehand technique and to compare revision surgery rates among three different imaging verification pathways. Summary of Background Data: Studies comparing different imaging modalities in freehand screw placement surgery are limited. Materials and Methods: A single-institution retrospective chart review identified adult patients who underwent freehand pedicle screw placement in the thoracic, lumbar or sacral levels. Patients were stratified into three cohorts based on the intraoperative imaging modality used to assess the accuracy of screw position: intraoperative X-rays (cohort 1); intraoperative O-arm (cohort 2); or intraoperative computed tomography (CT)-scan (cohort 3). Postoperative CT scans were performed on all patients in cohorts 1 and 2. Postoperative CT scan was not required in cohort 3. Screw accuracy was assessed using the Gertzbein-Robbins grading system. Results: A total of 9179 pedicle screws were placed in the thoracic or lumbosacral spine in 1311 patients. 210 (2.3%) screws were identified as Gertzbein-Robbins grades C-E on intraoperative/postoperative CT scan, 137 thoracic screws, and 73 lumbar screws (P<0.001). Four hundred and nine patients underwent placement of 2754 screws followed by intraoperative X-ray (cohort 1); 793 patients underwent placement of 5587 screws followed by intraoperative O-arm (cohort 2); and 109 patients underwent placement of 838 screws followed by intraoperative CT scan (cohort 3). Postoperative CT scans identified 65 (2.4%) and 127 (2.3%) malpositioned screws in cohorts 1 and 2, respectively. Eleven screws (0.12%) were significantly malpositioned and required a second operation for screw revision. Nine patients (0.69%) required revision operations: eight of these patients were from cohort 1 and one patient was from cohort 2. Conclusion:When compared to intraoperative X-ray, intraoperative O-arm verification decreased the revision surgery rate for malpositioned screws from 0.37% to 0.02%. In addition, our analysis suggests that the use of intraoperative O-arm can obviate the need for postoperative CT scans.

STUDY DESIGN:Systematic review. OBJECTIVES:To review the existing literature of prediction models in degenerative spinal surgery. METHODS:Review of PubMed/Medline and Embase databases was conducted to identify articles between January 1, 2000 and March 1, 2020 that reported prediction model performance for outcomes following elective degenerative spine surgery. RESULTS:Thirty-one articles were included. Twenty studies were of thoracolumbar, 5 were of cervical, and 6 included all spine patients. Five studies were externally validated. Prediction models were developed using machine learning (42%) and logistic regression (42%) as well as other techniques. Web-based calculators were included in 45% of published articles. Various outcomes were investigated, including complications, infection, length of stay, discharge disposition, reoperation, readmission, disability score, back pain, leg pain, return to work, and opioid dependence. CONCLUSIONS:Significant heterogeneity exists in methods used to develop prediction models of postoperative outcomes after degenerative spine surgery. Most internally validate their scores, but a few have been externally validated. Areas under the curve for most models range from 0.6 to 0.9. Techniques for development are becoming increasingly sophisticated with different machine learning tools. With further external validation, these models can be deployed online for patient, physician, and administrative use, and have the potential to optimize outcomes and maximize value in spine surgery.

Objective: To describe the results of a single-surgeon series and systematically review the literature on cementaugmented instrumented fusion with fenestrated pedicle screws. Methods: All patients treated by the senior surgeon using fenestrated screws between 2017 and 2019 with a minimum of 6-months of clinical and radiographic follow-up were included. For the systematic review, we used PRISMA guidelines to identify all prior descriptions of cement-augmented instrumented fusion with fenestrated pedicle screws in the English literature. Endpoints of interest included hardware loosening, cement leakage, and pulmonary cement embolism (PCE). Results: Our series included 38 patients (mean follow-up 14.8 months) who underwent cement-augmented instrumentation for tumor (47.3%), deformity/degenerative disease (39.5%), or osteoporotic fracture (13.2%). Asymptomatic screw lucency was seen in 2.6%, cement leakage in 445, and pulmonary cement embolism (PCE) in 5.2%. Our literature review identified 23 studies (n = 1526 patients), with low reported rates of hardware loosening (0.2%) and symptomatic PCE (1.0%). Cement leakage, while common (55.6%), produced symptoms in fewer than 1% of patients. Indications for cement-augmentation in this cohort included: spine metastasis with or without pathologic fracture (n = 18; 47.3%), degenerative spine disease or fixed deformity with poor underlying bone quality (n = 15; 39.5%), and osteoporotic fracture (n = 5; 13.2%).

Introduction: Hemangioblastomas typically present throughout the neuroaxis, particularly within the cerebellum and spinal cord. A rare case of cystic hemangioblastoma within the quadrigeminal cistern is described. Case Report: A 18-year-old female presented with 3 weeks of worsening headaches and vomiting. Workup revealed multiple contrast enhancing lesions throughout the neuroaxis concerning for hemangioblastoma, with the largest cystic mass within the quadrigeminal cistern causing brainstem compression and hydrocephalus. The patient experienced an acute neurologic decompensation likely due to brainstem compression and hydrocephalus, which was temporized by cyst drainage and endoscopic third ventriculostomy. After neurological stabilization and Von Hippel Lindau disease workup, she underwent a suboccipital craniotomy and resection of the hemangioblastoma via a transcystic approach. Intraoperatively, the trochlear nerve was identified and preserved. Conclusion: Operative approaches to the quadrigeminal cistern typically include the infratentorial supracerebellar approach and the occipital transtentorial approach. Here, we describe a safe and efficacious transcystic approach to a quadrigeminal cistern hemangioblastoma.

Thoracic meningoceles and dural ectasia are less commonly recognized manifestations of neurofibromatosis 1 (NF1). Rarely, large thoracic meningoceles may become compressive and lead to respiratory compromise secondary to lung compression. Surgical goals aim to increase lung aeration through decreasing the size of the meningocele through shunting, excision or repair of the meningocele, and varying degrees of dural tube reconstruction. There is no agreement on the best approach for large, symptomatic meningoceles. Here, we discuss the case of a 41-year-old woman with NF1 who presented with dyspnea and enlargement of a large, 19 cm thoracic meningocele. A multidisciplinary team of thoracic, plastic, and neurological surgery participated in the operation to excise the meningocele and reconstruct the dural tube without the need for subsequent shunting of spinal fluid. We also systematically review the literature on thoracic meningoceles in NF1 to understand the optimal treatment of this pathology.