A curious cervical spine case: multiple, primary CNS leiomyosarcomas presenting with rapid growth in the immunocompromised patient.

INTRODUCTION: Primary CNS leiomyosarcomas are rare, dural-based intracranial or intravertebral tumors seen in immunocompromised patients and are associated with latent EBV infection. They may mimic a meningioma or schwannoma on imaging but their clinical presentation progresses much more rapidly. Often times, these tumors are hard to distinguish from secondary, metastatic leiomyosarcoma. CASE PRESENTATION: A 30-year-old female with congenital HIV presented to clinic with shoulder pain, paresthesias of the right upper extremity and gait instability. She was noted to have a contrast enhancing dural-based spinal canal lesion measuring 1.5 cm at the C1 vertebral level on MRI. Surgery was proposed but patient deferred. She represented to our Emergency Department 1 month later with right-sided hemiparesis and difficulty with ambulation. On repeat MRI, the lesion had grown to 2.6 cm. She was taken to the OR emergently for gross total tumor resection. The histopathology demonstrated a primary CNS leiomyosarcoma. MRI scan of the brain revealed an extra-axial right frontal lobe lesion measuring 1.8 cm which was also treated with subtotal surgical resection followed by proton beam radiotherapy. DISCUSSION: Primary CNS leiomyosarcomas should be considered in young immunocompromised patients presenting with dural-based spinal cord tumors. Histopathological studies including EBV testing can definitively make the diagnosis. These tumors have an aggressive nature and need to be treated with complete surgical resection to prevent severe neurological deterioration and adjuvant therapy to prevent recurrence.

The role of venous anatomy in guiding treatment approach for dural arteriovenous fistulas of the craniocervical junction; case series & systematic review.

BACKGROUND: Dural arteriovenous fistulas (DAVF) of the craniocervical junction (CCF) are an uncommon entity with the following venous drainage pattern: inferior, superior and mixed. Patients may present with subarachnoid hemorrhage, myelopathy or brainstem dysfunction. CCJ DAVF can be treated with microsurgery or with transarterial and transvenous embolization, depending on the venous drainage pattern. We present our institutional experience of treating CCJ DAVFs along with a systematic review of the literature. METHODS: Six patients with CCJ DAVF were treated at our institution over five years. Data was collected using electronic medical record review. Systematic review was performed on CCJ DAVF using the PubMed database from 1990 to 2021. We characterized venous drainage patterns, treatment choices, and outcomes to create a classification system. RESULTS: 50 case reports, consisting of 115 patients, were included in our review. 61 (53.0 %) patients had inferior drainage while 32 (27.8 %) patients had superior drainage and 22 (19.2 %) patients had mixed venous drainage. Patients with inferior drainage had the fistulous connection at the foramen magnum while patients with superior drainage had a fistulous connection at C1-C2 (p value = 0.026). Patients with inferior drainage were more likely to present with myelopathy while patients with superior drainage presented with hemorrhage (p value = 0.000). CONCLUSIONS: Classifying the venous drainage pattern is essential in making treatment decision. Transvenous embolization works best with large superior venous drainage. If endovascular treatment is not an option, then surgical clipping can achieve successful cure. Transarterial embolization is a reasonable option in cases with a large arterial feeder.

Gram-Negative Ventriculostomy-Associated Infections Predict Shunt Dependency in Stroke Diagnoses and Other Brain Injuries.

BACKGROUND: Several predictors have been studied for shunt dependency after stroke and other brain injuries. However, little is known about the association between ventriculostomy-associated infections (VAIs) and impaired cerebrospinal fluid (CSF) outflow. Moreover, gram-negative (GN) VAIs induce a potent neuroinflammatory process and are clinically challenging to treat. OBJECTIVE: To assess if GN-VAIs predict ventriculoperitoneal shunt (VPS) dependency. METHODS: Retrospective analysis of postprocedure infection rates was performed in 586 patients with external ventricle drainage (EVD) placed on site between 2012 and 2018. We collected sex, age, stroke and nonstroke related, location of EVD placement, type of hospital, EVD duration, and EVD exchange. RESULTS: Among 586 patients requiring an EVD, 55 developed a VAI. Most were caused by gram-positive (GP) pathogens (61.8%). A total of 120 patients required a conversion from EVD to VPS. Patients with VAIs had higher rates of VPS placement (49.09% vs 17.65%, P < .001), whereas patients with GN-VAIs had significantly higher rates of EVD conversion to VPS (77.78% vs 35.29%, P = .012) compared with GP-VAIs. The multivariate analysis showed that GN-VAIs were an independent predictor for shunt dependency (odds ratio = 12.896; 95% CI 3.407-48.82, P < .001). In receiver operating characteristics analysis, those less than 44.5 yr of age and more than 12 d of EVD duration were identified as the best cutoff values to discriminate the development of GN-VAI. CONCLUSION: Patients who experience a GN VAI are in greater risk of impaired CSF outflow, thus requiring VPS placement.

Trans-Carotid and Trans-Radial Access for Mechanical Thrombectomy for Acute Ischemic Stroke: A Systematic Review and Meta-Analysis.

Objective We aim to demonstrate the safety and effectiveness of extra-femoral endovascular access for mechanical thrombectomy for acute ischemic stroke patients whose vascular anatomy precludes safe or maneuverable trans-femoral access. Methods Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to conduct a systematic review and meta-analysis with articles published until March 2018. The search protocol, including research questions and inclusion and exclusion criteria, were developed a priori. Our own institutional retrospective data were included in the cohort of case series. Results Eleven studies including 51 patients were included. Age ranged from 4th to 10th decade of life (average: 9.3rd decade) and 40.1% received IV tissue plasminogen activator. Initial National Institutes of Health Stroke Scale (NIHSS) score ranged from 1 to 36, (average: 17.6). Of the 51 patients, 39 (76%) patients suffered from anterior circulation large vessel occlusions versus 12 (24%) from posterior circulation occlusions. Site of access included 26 (51%) radial artery punctures, 23 (45%) direct percutaneous cervical carotid punctures, 1 brachial artery puncture, and 1 direct extradural vertebral artery puncture. Technical success was achieved in 43/51 (84%) of patients. The average modified Rankin Scale at discharge was 2.93 (n=26). There were no complications in 25 patients who underwent radial arterial access. Two (7.4%) of 27 cervical access patients developed hematoma. Conclusions Trans-carotid and trans-radial access for intervention in acute ischemic stroke is safe and effective. There may be instances in which these approaches should be considered first line before standard femoral approaches.

Virtual reality simulation of neuroendovascular intervention improves procedure speed in a cohort of trainees.

BACKGROUND: Realistic virtual reality (VR) simulators have greatly expanded the tools available for training surgeons and interventionalists. While this technology is effective in improving performance in many fields, it has never been evaluated for neuroendovascular procedures. This study aims to determine whether VR is an effective tool for improving neuroendovascular skill among trainees. METHODS: Trainees performed two VR revascularizations of a right-sided middle cerebral artery (MCA) thrombosis and their times to procedural benchmarks (time to enter internal carotid artery [ICA], traverse clot, and complete procedure) were compared. To determine whether the improvement was case specific, trainees with less procedural exposure were timed during VR left-sided ICA (LICA) aneurysm coiling before or after performing MCA thrombectomy simulations. To determine the value of observing simulations, medical students were timed during the right MCA revascularization simulations after watching other VR procedures. RESULTS: Trainees significantly improved their time to every procedural benchmark during their second MCA revascularization (mean decrease = 1.08, 1.57, and 2.24 min; P = 0.0072, 0.0466, and 0.0230). In addition, time required to access the LICA during aneurysm coiling was shortened by 0.77 min for each previous VR right MCA revascularization performed (P = 0.0176; r 2 = 0.71). Finally, medical students' MCA revascularization simulation times improved by 0.87 min for each prior simulation viewed (P < 0.0221; r 2 = 0.96). CONCLUSION: Both performance and viewing of simulated procedures produced significant decreases in time to reach neuroendovascular procedural benchmarks. These data show that VR simulation is a valuable tool for improving trainee skill in neuroendovascular procedures.

Contemporary Updates on Clinical Trials of Antiangiogenic Agents in the Treatment of Glioblastoma Multiforme.

Glioblastoma multiforme (GBM) has the highest rate of vascular proliferation among solid tumors. Angiogenesis is the central feature of rapid tumor growth in GBM and therefore remains an appealing therapeutic target in the treatment of these highly malignant tumors. Antiangiogenic therapy is emerging as an important adjuvant treatment. Multiple antiangiogenic agents targeting various sites in vascular endothelial growth factor (VEGF) and integrin pathways have been tested in clinical trials of newly diagnosed and recurrent GBMs. These include bevacizumab, enzastaurin, aflibercept, cediranib, and cilengitide. In this review, we discuss the current status and challenges facing clinical application of antiangiogenic treatment including anti-VEGF therapy and integrin pathway agents' therapy in glioblastoma. Here, we highlight a strong biologic rationale for this strategy, also focusing on integrin pathways. PubMed-indexed clinical trials published in English on antiangiogenic treatment of glioblastomas in the past 5 years were reviewed. The results of the current clinical trials of these agents are presented.

Trends in the Use of Bone Morphogenetic Protein-2 in Adult Spinal Deformity Surgery: A 10-Year Analysis of 54 054 Patients.

BACKGROUND: Bone morphogenetic protein-2 (BMP-2) is an available bone graft option in spinal fusion surgery. The purpose of this study is to investigate the trends of BMP-2 utilization in adult spinal deformity (ASD) surgery. METHODS: The Nationwide Inpatient Sample database from 2002 to 2011 was reviewed. Inclusion criteria were patients over 18 years of age who underwent spinal fusion for ASD. Trends of BMP-2 use were examined over time, as well as stratified based on patient and surgical characteristics. All analyses were done after application of discharge weights to produce national estimates. RESULTS: There were 54 054 patients who met inclusion criteria and were included in this study. The overall rate of BMP-2 use was 39.7% (95% confidence interval 35.0%- 44.3%). Overall, there was steady increase in its use over time, with the highest peak in 2009 (55.3% of all cases used BMP-2), and then a decrease up to 37.9% in 2011 (P < .001). The rate of BMP-2 use was significantly higher for patients older than 54 years of age (compared to patients <54, P < .001). It was also higher in females (P = .009), Caucasian patients (P = .006), and Medicare patients (P = .006). Its use was 28.6% in the Northeast, 38.1% in the South, 45.2% in the Midwest, and 48.2% in the West (P = .035). Circumferential procedures had the highest rate of BMP-2 use (44.3%, P = .045). Average total hospital charges were $152,403 ± 117,454 for patients who did not receive BMP-2 and $205,426 ± 137,561 for patients who did (P < .001). CONCLUSION: After analysis of a large nationwide database, it was found that the rate of BMP-2 use in ASD surgery is approximately 40%. There was a significant increase in use from 2002 to 2009, and a decrease thereafter. The highest rates of use were found in older patients, female patients, white patients, Medicare patients, circumferential approaches, and patients undergoing surgery in the Midwest and West regions.

Risk Factors for 30-Day Readmissions and Reoperations After 3-Column Osteotomy for Spinal Deformity.

STUDY DESIGN: Retrospective study of a prospectively collected database. OBJECTIVE: To investigate the rate and risk factors for 30-day readmissions and reoperations after 3-column osteotomy (3CO). METHODS: The American College of Surgeons National Surgical Quality Improvement Program database (2012-2014) was reviewed. Inclusion criteria were adult patients who underwent 3CO. The rate of 30-day readmission/reoperation was examined, and the association between patient/operative characteristics and outcome was investigated via multivariate analysis. RESULTS: There were 299 patients who underwent a 3CO for spinal deformity. The rate of 30-day readmission and reoperation was 11.0% and 8.4%, respectively; 7.7% of readmissions were related to the primary procedure and 3.3% were unrelated. The most common unique cause for readmission was wound infection in 27.2% of cases. Among reoperations, the most common unique indications were wound infection (20.0%) and implant-related complications (20.0%). On multivariate analysis, obesity (odds ratio [OR] = 2.96; 95% CI = 1.06-8.25; P = .038), chronic obstructive pulmonary disease (OR = 20.8; 95% CI = 3.49-123.5; P = .001), and fusion of 13 or more spinal levels were independent predictors of readmission (OR = 4.86; 95% CI = 1.21-19.5; P = .025). On the other hand, independent predictors of reoperation included chronic obstructive pulmonary disease (OR = 6.33; 95% CI = 1.16-34.5; P = .033) and chronic steroid use (OR = 6.69; 95% CI = 1.61-27.7; P = .009). CONCLUSION: Wound complications and short-term implant-related complications are important causes of readmission and/or reoperation after 3CO. Preoperative factors such as obesity, chronic lung disease, chronic steroid use, and long-segment fusion procedures may significantly increase the risk of 30-day morbidity following high-grade osteotomies.

Greater Cortical Thickness Is Associated With Enhanced Sensory Function After Arm Rehabilitation in Chronic Stroke.

OBJECTIVE: Somatosensory function is critical to normal motor control. After stroke, dysfunction of the sensory systems prevents normal motor function and degrades quality of life. Structural neuroplasticity underpinnings of sensory recovery after stroke are not fully understood. The objective of this study was to identify changes in bilateral cortical thickness (CT) that may drive recovery of sensory acuity. METHODS: Chronic stroke survivors (n = 20) were treated with 12 weeks of rehabilitation. Measures were sensory acuity (monofilament), Fugl-Meyer upper limb and CT change. Permutation-based general linear regression modeling identified cortical regions in which change in CT was associated with change in sensory acuity. RESULTS: For the ipsilesional hemisphere in response to treatment, CT increase was significantly associated with sensory improvement in the area encompassing the occipital pole, lateral occipital cortex (inferior and superior divisions), intracalcarine cortex, cuneal cortex, precuneus cortex, inferior temporal gyrus, occipital fusiform gyrus, supracalcarine cortex, and temporal occipital fusiform cortex. For the contralesional hemisphere, increased CT was associated with improved sensory acuity within the posterior parietal cortex that included supramarginal and angular gyri. Following upper limb therapy, monofilament test score changed from 45.0 ± 13.3 to 42.6 ± 12.9 mm ( P = .063) and Fugl-Meyer score changed from 22.1 ± 7.8 to 32.3 ± 10.1 ( P < .001). CONCLUSIONS: Rehabilitation in the chronic stage after stroke produced structural brain changes that were strongly associated with enhanced sensory acuity. Improved sensory perception was associated with increased CT in bilateral high-order association sensory cortices reflecting the complex nature of sensory function and recovery in response to rehabilitation.

Predicting critical care unit-level complications after long-segment fusion procedures for adult spinal deformity.

BACKGROUND: To identify predictive factors for critical care unit-level complications (CCU complication) after long-segment fusion procedures for adult spinal deformity (ASD). METHODS: The American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database [2010-2014] was reviewed. Only adult patients who underwent fusion of 7 or more spinal levels for ASD were included. CCU complications included intraoperative arrest/infarction, ventilation >48 hours, pulmonary embolism, renal failure requiring dialysis, cardiac arrest, myocardial infarction, unplanned intubation, septic shock, stroke, coma, or new neurological deficit. A stepwise multivariate regression was used to identify independent predictors of CCU complications. RESULTS: Among 826 patients, the rate of CCU complications was 6.4%. On multivariate regression analysis, dependent functional status (P=0.004), combined approach (P=0.023), age (P=0.044), diabetes (P=0.048), and surgery for over 8 hours (P=0.080) were significantly associated with complication development. A simple scoring system was developed to predict complications with 0 points for patients aged <50, 1 point for patients between 50-70, 2 points for patients 70 or over, 1 point for diabetes, 2 points dependent functional status, 1 point for combined approach, and 1 point for surgery over 8 hours. The rate of CCU complications was 0.7%, 3.2%, 9.0%, and 12.6% for patients with 0, 1, 2, and 3+ points, respectively (P<0.001). CONCLUSIONS: The findings in this study suggest that older patients, patients with diabetes, patients who depend on others for activities of daily living, and patients who undergo combined approaches or surgery for over 8 hours may be at a significantly increased risk of developing a CCU-level complication after ASD surgery.

Virtual modeling, stereolithography, and intraoperative CT guidance for the optimization of sagittal synostosis reconstruction: a technical note.

INTRODUCTION: Sagittal synostosis affects 1 in 1000 live births and may result in increased intracranial pressure, hindrance of normal neural development, and cosmetic deformity due to scaphocephaly. Historically, several approaches have been utilized for surgical correction and recently, computed tomography (CT)-guided reconstruction procedures are increasingly used. In this report, the authors describe the use of a CT-derived virtual and stereolithographic (3D printed) craniofacial models, which are used to guide intraoperative bone placement, and intraoperative CT guidance for confirmation of bone placement, to ensure the accuracy of surgical correction of scaphocephaly, as demonstrated to parents. METHODS: Preoperative high-resolution CT imaging was used to construct 3D image sets of the skulls of two infants (a 14-month-old female and a 6-month-old male) with scaphocephaly. These 3D image sets were then used to create a virtual model of the proposed surgical correction for each of the infants' deformities, which was then printed and made available for use intraoperatively to plan the bone flap, fashion the bone cuts, and optimize graft placement. After the remodeling, adherence to the preoperative plan was assessed by overlaying a CT scan of the remodeled skull with the virtual model. Deviations from the preoperative model were noted. RESULTS: Both patients had excellent postoperative cosmetic correction of head shape and contouring. The mean operative time was 5 h, blood loss was 100 ml, and one child required modification of the subocciput after intraoperative imaging showed a deviation of the reconstruction from the surgical goal as depicted by the preoperative model. CONCLUSION: The addition of neuro-navigation to stereolithographic modeling ensured the accuracy of the reconstruction for our patients and provided greater confidence to both surgeons and parents. While unisutural cases are presented for clarity, correction was still required for one patient. The cost of the models and the additional CT required must be weighed against the complexity of the procedure and possibly reserved for patients with potentially complicated corrections.

Flow-Diverting Stents for the Obliteration of Symptomatic, Infectious Cavernous Carotid Artery Aneurysms.

BACKGROUND: Intracavernous aneurysms constitute up to 9% of all intracranial aneurysms and 6% are infectious (IIA). First line therapy is a protracted antibiotic course, yet with failure, surgery and endovascular parent vessel sacrifice have been utilized. Reconstructive endovascular therapies have emerged for aneurysm control and may demonstrate a safer therapeutic alternative. OBJECTIVE: To present an IIA treated with a flow-diverting Pipeline stent (ev3 Neurovascular, Irvine, California). METHODS: A 41-yr-old female presented with visual loss, ophthalmoplegia, and cavernous sinus thrombosis with an associated phlegmon. Transsphenoidal evacuation was performed without complication or bleeding and she continued on medical therapy. Two weeks postoperatively, she developed a worsening right third cranial nerve palsy and MRA demonstrated a 1-cm right IIA, not evident on postoperative MRI. Three days of dual antiplatelet therapy preceded successful pipeline embolization. Angiography demonstrated aneurysm obliteration at 3 mo and her right ophthalmoplegia resolved. RESULTS: A literature review identified 6 reported cases of IIAs treated with stent embolization. Only 1 documented a flow-diverting Silk stent used in a child. All lesions were obliterated at follow-up without neurological sequelae. No complication arose with implantation in the setting of infection, and as few as 3 d of dual antiplatelet therapy was sufficient for preprocedural prophylaxis, although in Vivo antiplatelet activity may be more significant. CONCLUSION: We report the first case of an IIA treated with a flow-diverting pipeline stent. These devices preserve native vasculature and neurological function compared to surgical and endovascular vessel sacrifice strategies. They appear to be safe management options for the treatment of IIAs.

Glioblastoma multiforme presenting with an open ring pattern of enhancement on MR imaging.

BACKGROUND: Intracerebral ring enhancing lesions can be the presentation of a variety of pathologies, including neoplasia, inflammation, and autoimmune demyelination. Use of a precise diagnostic algorithm is imperative in correctly treating these lesions and minimizing potential adverse treatment effects. CASE DESCRIPTION: A 55-year-old patient presented to the hospital with complaints of a post-concussive syndrome and a non-focal neurologic exam. Imaging revealed a lesion with an open ring enhancement pattern, minimal surrounding vasogenic edema, and minimal mass effect. Given the minimal mass effect, small size of the lesion, and nonfocal neurological exam, we elected to pursue a comprehensive noninvasive neurologic workup because our differential ranged from inflammatory/infectious to neoplasm. Over the next 8 weeks, the patient's condition worsened, and repeat imaging showed marked enlargement of the lesion with a now closed ring pattern of enhancement with satellite lesions and a magnetic resonance (MR) spectroscopy and perfusion signature suggestive of neoplasm. The patient was taken to surgery for biopsy and debulking of the lesion. Surgical neuropathology examination revealed glioblastoma multiforme. CONCLUSION: The unique open ring enhancement pattern of this lesion on initial imaging is highly specific for a demyelinating process, however, high-grade glial neoplasms can also present with complex and irregular ring enhancement including an open ring sign. Therefore, other imaging modalities should be used, and close follow-up is warranted when the open ring sign is encountered.

Use of Google Glass to Enhance Surgical Education of Neurosurgery Residents: "Proof-of-Concept" Study.

BACKGROUND: The relatively decreased time spent in the operating room and overall reduction in cases performed by neurosurgical trainees as a result of duty-hour restrictions demands that the pedagogical content within each surgical encounter be maximized and crafted toward the specific talents and shortcomings of the individual. It is imperative to future generations that the quality of training adapts to the changing administrative infrastructures and compensates for anything that may compromise the technical abilities of trainees. Neurosurgeons in teaching hospitals continue to experiment with various emerging technologies-such as simulators and virtual presence-to supplement and improve surgical training. METHODS: The authors participated in the Google Glass Explorer Program in order to assess the applicability of Google Glass as a tool to enhance the operative education of neurosurgical residents. Google Glass is a type of wearable technology in the form of eyeglasses that employs a high-definition camera and allows the user to interact using voice commands. RESULTS: Google Glass was able to effectively capture video segments of various lengths for residents to review in a variety of clinical settings within a large, tertiary care university hospital, as well as during a surgical mission to a developing country. The resolution and quality of the video were adequate to review and use as a teaching tool. CONCLUSION: While Google Glass harbors the potential to dramatically improve both neurosurgical education and practice in a variety of ways, certain technical drawbacks of the current model limit its effectiveness as a teaching tool.

Cortical herniation through compressive subdural membrane in an infant with a history of a large bihemispheric subdural hematoma and subdural-peritoneal shunt: case report.

Cortical herniation through subdural membrane formation is a rare complication of chronic subdural fluid collections and may occur following subdural shunting. The authors present a unique case of progressive cortical herniation through a compressive subdural membrane that occurred concomitant with a functioning subdural-peritoneal shunt.