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OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors found throughout the body, with their clinical course in children still not completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this population from a large national database. METHODS All patients with MPNSTs aged ≤ 18 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 251 pediatric patients with MPNSTs (132 [53%] females and 119 [47%] males) were identified; the mean age at diagnosis was 13.1 years (range 1–18 years). There were 84 (33%) MPNSTs located in the extremities, 127 (51%) were smaller than 1 cm, and 22 (9%) had metastasis at the time of diagnosis. In terms of treatment, surgery was pursued in 187 patients (74%), chemotherapy in 116 patients (46%), and radiation therapy in 129 patients (61%). The 5-year overall survival rate was estimated at 52% (95% CI 45%–59%), with a median survival of 64 months (range 36–136 months). Multivariate regression revealed that older age (HR 1.10, p < 0.01), metastases at the time of diagnosis (HR 2.14, p = 0.01), and undergoing biopsy only (HR 2.98, p < 0.01) significantly and independently predicted a shorter overall survival. Chemotherapy and radiation therapy were not statistically significant. CONCLUSIONS In this study, the authors found that older patient age, tumor metastases at the time of diagnosis, and undergoing only biopsy significantly and independently predicted poorer outcomes. Only approximately half of patients survived to 5 years. These results have shown a clear survival benefit in pursuing maximal safe resection in pediatric patients with MPNSTs. As such, judicious workup with meticulous resection by an expert team should be considered the standard of care for these tumors in children.

OBJECTIVE The objective of this study was to determine the frequency with which brain biopsy for presumed CNS relapse of systemic hematological malignancies yields new, actionable diagnostic information. Hematological malignancies represent a disparate group of genetic and histopathological disorders. Proclivity for brain involvement is dependent on the unique entity and may occur synchronously or metasynchronously with the systemic lesion. Diffuse large B-cell lymphomas (DLBCLs) have a high propensity for brain involvement. Patients in remission from systemic DLBCL may present with a lesion suspicious for brain relapse. These patients often undergo brain biopsy. The authors’ a priori hypothesis was that brain biopsy in patients with a history of systemic DLBCL and a new brain MRI lesion would have lower diagnostic utility compared with patients with non-DLBCL systemic malignancies. METHODS The authors performed a retrospective review of patients who underwent brain biopsy between 2000 and 2019. Inclusion criteria were patients ≥ 18 years of age with a prior systemic hematological malignancy in remission presenting with a new brain MRI lesion concerning for CNS relapse. Patients with a history of any CNS neoplasms, demyelinating disorders, or active systemic disease were excluded. The main outcome was the proportion of patients with a distinct histopathological brain diagnosis compared with the systemic malignancy. The authors secondarily assessed overall survival, procedure-related morbidity, and 30-day mortality. RESULTS Sixty patients met inclusion criteria (40 males and 20 females); the median age at brain biopsy was 67 years (range 23–88 years). The median follow-up was 8.5 months (range 0.1–231 months). Thirty-nine (65.0%) patients had DLBCL and 21 (35%) had non-DLBCL malignancies. Thirty-five of 36 (97.2%) patients with prior systemic DLBCL and a diagnostic biopsy had histopathological confirmation of the original systemic disease versus 0 of 21 patients with non-DLBCL systemic malignancies (p < 0.001). Morbidity and 30-day mortality were 8.3% and 10.0%, respectively; 2 of 6 30-day mortalities were directly attributable to the biopsy. The median overall survival following brain biopsy was 10.8 months. CONCLUSIONS Patients with a history of systemic DLBCL and presumed CNS relapse gained minimal clinical benefit from brain biopsy but were at high risk of morbidity and mortality. In patients with a history of non-DLBCL systemic malignancies, brain biopsy remained critical given the high likelihood for discovery of distinct diagnostic entities. It was determined that patients with a prior systemic DLBCL and presumed brain relapse should likely receive empirical therapy obviating treatment delay and the risks of brain biopsy.

BACKGROUND The diagnosis of glioblastoma (GBM) in infants aged ≤ 1 year is extremely rare, and its comparability to the more common adult diagnosis is underexplored. Correspondingly, the objective of this study was to interrogate a national cancer database to elucidate the typical survival and clinical profile of this demographic. METHODS All GBM patients aged ≤ 1 year in the U.S. National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival (OS) was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 86 patients satisfied criteria for entry into study, making up 0.08% of all GBM diagnoses in the database. There were 32 (37%) females and 54 (63%) males. Irrespective of treatment, median OS was 67.3 months (95% CI, 46-91), which was distinct from all other ages and pediatric age groups. There were 74 (86%) treated by surgery, 51 (59%) treated by chemotherapy, and 17 (20%) treated by radiation therapy. Multivariable analysis demonstrated that Hispanic status (HR = 3.41, P = 0.02) and the presence of comorbidity (HR = 3.24, P = 0.01) independently predicted shorter OS, whereas treatment with chemotherapy (HR = 0.18, P

Pineal parenchymal tumors of intermediate differentiation (PPTID) are a rare group of pineal parenchymal tumors classified by histology as either World Health Organization (WHO) Grades 2 or 3. The rarity of these tumors in adults has left a number of clinical management questions open. Correspondingly, the aim of this study was to aggregate a large PPTID cohort with sufficient statistical power from a large national cancer database to analyze prognostic parameters. All PPTID patients aged over 18 years in the U.S. National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized and survival was modeled using Kaplan–Meier and Cox regression analyses. A total of 103 adult PPTID patients were identified in the NCDB with 63 (61%) WHO Grade 2 and 40 (39%) WHO Grade 3 tumors. Overall, mean age was 53 ± 18 years with even gender distribution. A total of 75 (73%) patients underwent surgical resection for diagnosis, with gross total resection (GTR) was the most common resection outcome in 50/75 (67%). Chemotherapy was utilized in 18 (17%) patients, and radiation therapy in 37 (36%) patients. Overall, 5-year survival rate was estimated to be 54% (95% CI 42–64%), with mean survival was 84 (95% CI 69–99) months. Patients with Grade 2 tumors survived statistically longer than Grade 3 tumor counterparts (P

Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.

Cavernous sinus cavernous malformations (CSCMs) is a vascular malformation of the cavernous sinus. Nowadays, there is an increasing preference to withhold using the terms cavernoma or cavernous hemangioma in order to stop considering these lesions as vascular neoplasms. These lesions are highly vascularized making surgical resection a challenge, mainly in endoscopic approaches. We present a case of this tumor treated in our institution with an endoscopic endonasal approach and incomplete resection. Because of the strenuous resection through this approach, we systematically reviewed the reported endoscopic cases of CSCMs to determine their intraoperative complications, results and tumor features.Using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, one database (PubMed) and crossed references were queried for CSCMs from 1948 to 2020. Data regarding demographic features, clinical presentation, MRI features, surgical results and overall pathology features extracted.Eighteen patients were selected (including our case). The mean age was 50.4 ± 14 years. Pituitary dysfunction and cavernous sinus nerve compression were the most reported symptoms. Only five cases (27%) reported a gross total resection (GTR) through endoscopic endonasal approach. Intraoperative bleeding was the most frequent intraoperative complication.We present a comprehensive analysis of every reported CSCM treated through endoscopic approach. Partial or subtotal resection are the most used techniques because of the intraoperative bleeding and the adherence to surrounding structures. Radiotherapy is a very good option for patients with incomplete resections.

While laser ablation has become an increasingly important tool in the neurosurgical oncologist’s armamentarium, deep seated lesions, and those located near critical structures require utmost accuracy during stereotactic laser catheter placement. Robotic devices have evolved significantly over the past two decades becoming an accurate and safe tool for stereotactic neurosurgery. Here, we present our single center experience with the MedTech ROSA ONE Brain robot for robotic guidance in laser interstitial thermal therapy (LITT) and stereotactic biopsies. We retrospectively analyzed the first 70 consecutive patients treated with ROSA device at a single academic medical center. Forty-three patients received needle biopsy immediately followed by LITT with the catheter placed with robotic guidance and 27 received stereotactic needle biopsy alone. All the procedures were performed frameless with skull bone fiducials for registration. We report data regarding intraoperative details, mortality and morbidity, diagnostic yield and lesion characteristics on MRI. Also, we describe the surgical workflow for both procedures. The mean age was 60.3 ± 15 years. The diagnostic yield was positive in 98.5% (n = 69). Sixty-three biopsies (90%) were supratentorial and seven (10%) were infratentorial. Gliomas represented 54.3% of the patients (n = 38). There were two postoperative deaths (2.8%). No permanent morbidity related to surgery were observed. We did not find intraoperative technical problems with the device. There was no need to reposition the needle after the initial placement. Stereotactic robotic guided placement of laser ablation catheters and biopsy needles is safe, accurate, and can be implemented into a neurosurgical workflow.

Intraoperative pathologic diagnosis traditionally involves frozen section histopathology, which may be labor and time intensive. Indeed, a technique that streamlines the acquisition and evaluation of intraoperative histologic data may expedite surgical decision-making and shorten operative time. Stimulated Raman histology (SRH) is an emerging technology that allows for more rapid acquisition and interpretation of intraoperative histopathologic data.A blinded, prospective cohort study was performed for 82 patients undergoing resection for a central nervous system tumor. Of these, 21 patients were diagnosed with glioma either intraoperatively or postoperatively on permanent section histology and included in this study. Time to diagnosis (TTD) and diagnostic accuracy relative to permanent section (the gold standard) were compared between SRH-based diagnosis and conventional frozen section histology. Diagnostic concordance with permanent section was also compared between frozen histopathology and SRH diagnosis.Diagnostic accuracy was not significantly different between methods (P = 1.00). Diagnostic concordance was not significantly different between methods when comparing 95% confidence intervals for kappa values (κ = 0.215; κ = 0.297; κ = 0.369). Lastly, mean TTD was significantly shorter with SRH-based diagnosis compared with frozen section (43 vs. 9.7 minutes, P0.0001). SRH was able to identify key features associated with varying glioma types.SRH allows for rapid intraoperative diagnosis without sacrificing diagnostic accuracy. SRH may serve as a promising adjuvant to conventional histopathology to expedite intraoperative pathology consultation and surgical decision-making.

Atypical (WHO grade II) and malignant meningiomas (WHO Grade III) are a rare subset of primary intracranial tumors. Given their relatively high recurrence rate after surgical resection and radiotherapy, there has been a recent push to explore other adjuvant treatment options for these treatment-refractory tumors. Recent advances in molecular sequencing of tumors have elucidated new pathways and drug targets which are currently being studied. This article provides a thorough overview of novel investigational therapeutics including targeted therapy, immunotherapy, and new technological modalities for atypical and malignant meningiomas. We performed a comprehensive review of the available literature regarding preclinical and clinical evidence for emerging treatments for high grade meningiomas from 1980 to 2020 including contemporaneous clinical trials. There is encouraging preclinical evidence regarding the efficacy of the emerging treatments discussed in this article. Several clinical trials are currently recruiting patients to translate targeted molecular therapy for meningiomas. Several clinical studies have suggested a clinical benefit of combinatorial treatment for these treatment-refractory tumors. With numerous active clinical trials for high grade meningiomas, a meaningful improvement in the outcomes for these tumors may be on the horizon.

Background During the coronavirus 19 (COVID-19) pandemic, physicians have begun adapting their daily practices to prevent transmissions. In this study we aimed to provide surgical neuro-oncologists with practice guidelines during the COVID-19 pandemic based on objective data from a high-volume brain tumor surgeon at the current COVID-19 epicenter. Methods All outpatient visits and surgeries performed by the senior author during the COVID-19 pandemic were compared between the initial quarantine (3/23/20–5/4/20), the plateau period following quarantine (5/5/20–6/27/20), and the second peak (6/28/20–7/20/20). In-person and telemedicine visits were evaluated for crossovers. Surgeries were subdivided based on lesion type and evaluated across the same time period. Results From 3/23/20–7/20/20, 469 clinic visits and 196 surgeries were identified. After quarantine was lifted, face-to-face visits increased (P

Introduction: Infantile endodermal oculomotor nerve cyst (EONC) is an extremely rare entity. There are very few pediatric cases reported in the literature, and as expected, oculomotor palsy is the most common presenting symptom. To date however, the risk of recurrence of these lesions following surgical intervention is unclear due to a lack of long-term radiological follow-up. Case Presentation: We present a case of a 13-month-old male patient with an EONC and detail his surgical fenestration and postoperative course. Somewhat surprisingly, re-expansion occurred within 6 months and remained stable 2 years later. Discussion: A surgical approach to fenestration of an EONC in an infant is possible and should be performed by an expert neurosurgeon. Early recurrence is underreported in the current literature, and we encourage longer term radiological surveillance of these lesions after surgery to optimize primary and recurrent management in the future.

Atypical teratoid/rhabdoid tumor (ATRT) is a rare and largely pediatric diagnosis, with poor survival. Diagnosis below the age of 3 years is characteristically seen as a poor prognostic sign. However, elucidating if clinical differences exist within this niche age group has never been attempted before. Correspondingly, we sought to characterize clinical profile of ATRT diagnoses before the age of 3 years based on separate ages of diagnosis. All pediatric ATRT patients aged < 3 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Age groups were divided based on diagnoses at ages 0–1 years in group 1, 1–2 years in group 2, and 2–3 years in group 3. Data were summarized, and overall survival (OS) was modeled using Kaplan–Meier and Cox regression analyses. A total of 354 ATRT diagnoses were made before the age of 3 years, with surgery used in 316 (89%) cases, chemotherapy in 242 (68%) cases, and radiation therapy in 118 (33%) cases. In terms of diagnosis age, there were 153 (43%) in group 1, 137 (39%) in group 2, and 64 (18%) in group 3. With respect to OS, median value was 9.9 months in group 1, 28.4 months in group 2, and 15.9 months in group 3. Upon multivariate analysis, receiving radiation therapy was the only parameter shared amongst all three groups as independently prognostic of longer OS (HR 0.53, P = 0.01 in group 1; HR 0.34, P < 0.01 in group 2; HR 0.31, P < 0.01 in group 3). In group 1, surgery (HR 0.47, P < 0.01) and chemotherapy (HR 0.44, P < 0.01) were also independently prognostic of longer OS. In group 3, multiple socioeconomic parameters were identified to independently predict longer OS. There were no additional predictive parameters identified in group 2. Although ATRT diagnosed before the age of 3 is typically viewed a poor prognostic age category, our findings demonstrate that the clinical profile of this pediatric niche is highly heterogeneous based on age of diagnosis. Survival of only those diagnosed between 0 and 1 years is independently prognosticated by all three treatment modalities; patients diagnosed between 1 and 2 years trend towards longest survival, and socioeconomic parameters are most influential in those diagnosed between 2 and 3 years.

OBJECTIVEGlioblastoma (GBM) during infancy is rare, and the clinical outcomes of congenital GBM are not well understood. Correspondingly, the aim of this study was to present a long-term survivor case from the authors’ institution, and establish an integrated cohort of cases across the published literature to better understand the clinical course of this disease in this setting.METHODSThe authors report the outcomes of an institutional case of congenital GBM diagnosed within the first 3 months of life, and performed a comprehensive literature search for published cases from 2000 onward for an integrated survival analysis. All cases were integrated into 1 cohort, and Kaplan-Meier estimations, Fisher’s exact test, and logistic regression were used to interrogate the data.RESULTSThe integrated cohort of 40 congenital GBM cases consisted of 23 (58%) females and 17 (42%) males born at a median gestational age of 38 weeks (range 22–40 weeks). Estimates of overall survival (OS) at 1 month was 67%, at 1 year it was 59%, and at 10 years it was 45%, with statistically superior outcomes for subgroups in which patients survived to be treated by resection and chemotherapy. In the overall cohort, multivariable analysis confirmed resection (p < 0.01) and chemotherapy (p < 0.01) as independent predictors of superior OS. Gestational age > 38 weeks (p < 0.01), Apgar scores ≥ 7 at 5 minutes (p < 0.01), absence of prenatal hydrocephalus (p < 0.01), and vaginal delivery (p < 0.01) were associated with greater odds of surgical diagnosis versus autopsy diagnosis.CONCLUSIONSCongenital GBM can deviate from the expected poor prognosis of adult GBM in terms of OS. Both resection and chemotherapy confer statistically superior prognostic advantages in those patients who survive within the immediate postnatal period, and should be first-line considerations in the initial management of this rare disease.

Although recurrent anaplastic ependymoma in pediatric patients is not uncommon, recurrent disease leading to widespread metastases to the peritoneum is extremely rare. We present a case of an 18-month old male who initially presented with posterior fossa anaplastic ependymoma, who then proceeded to present 1 year later with spinal recurrence, and then 2 years after that with widespread disease involving the intracranial ventricular system and peritoneum. We posit that surgical interventions to treat primary and recurrent presentations in combination with a conduit to the peritoneum via a ventriculoperitoneal shunt contributed to the mechanisms of this complex case.

Treatment of non-saccular aneurysms of the posterior circulation poses a great challenge with unpredictable outcomes due to the absence of a true aneurysm neck and the presence of perforating vessels. In this article, we aim to compare endovascular treatment of unruptured posterior circulation non-saccular aneurysms with stent-assisted coiling (SAC) and flow diversion (FD) in terms of occlusion rate and clinical outcomes. A systematic search of electronic databases from inception to August 2019 identified 484 articles for screening. After proper inclusion/exclusion criteria, 15 articles were included and data were extracted and analyzed using meta-analysis of proportions. The pooled cohort consisted of 430 aneurysms: 128 (29.7%) treated with SAC in 5 studies and 302 (70.3%) treated with FD in 11 studies. Complete/near-complete occlusion was achieved in 83% after FD (95% CI 0.75 to 0.90; I2=45%) and 84% after SAC (95% CI 0.72 to 0.91; I2=22%), with no significant difference between techniques (p=0.95). Periprocedural complications were observed in 18% after FD (95% CI 0.14 to 0.23; I2=0%) and 6% after SAC (95% CI 0.02 to 0.13; I2=0%); the subgroup analysis was statistically significant (p=0.008). Furthermore, no statistically significant difference was observed in favorable clinical outcomes between groups. These results suggest similar efficacy in occlusion rate and favorable clinical outcome for posterior circulation non-saccular aneurysms treated with SAC and FD. Stroke was the most common complication regardless of treatment modality, and a lower periprocedural complication rate was noted with SAC. Further studies are needed with the primary focus of reducing the risk of stroke with either modality.

Departments of neurosurgery in the United States have had a long history of hosting international visitors for the advancement of their neurosurgical education. The goal of the present study was to determine the existence of a geographic profile of international visitors for education purposes, both students and graduates, to the United States through the anecdotal experience of 1 department to explore the reach and worth of hosting such opportunities.Administrative records were retrospectively reviewed for international visitors over the preceding 10 years, 2009-2019, were surveyed at 1 institution. Visitors were grouped as either medical students or graduates, visiting for clinical or research purposes. Geographic trends were compared using the Pearson chi-squared test.We identified 128 visitors from 33 countries during the study period. The most common home countries were Italy (10%), China (9%), and India (7%). The cohort involved 62 (48%) students and 66 (52%) graduates, and 59 (46%) and 69 (54%) visited for clinical and research purposes, respectively. The geographic distribution of student versus graduate visitors was statistically significant by region (P0.01). However, the geographic distribution of clinical versus research visitors was not statistically different by region (P = 0.62).There appears to be international appeal worldwide to participate in neurosurgical education in the United States. At our institution, every geographic region was represented in our most recent decade of experience. International visitor profiles appear to be associated with particular geographic patterns depending on their status as either a medical student or graduate.

The most recent cIMPACT-NOW update highlighted the homozygous deletion of the Cyclin Dependent Kinase Inhibitor 2A (CDKN2A) gene as a clinically important molecular alteration in IDH-mutant glioma. Correspondingly, we systematically reviewed the contemporary literature to affirm the contemporary stance of the literature on the prognostic significance of this alteration in this setting based on the current World Health Organization (WHO) Grade classification. A systematic search of seven electronic databases from inception to February 2020 was conducted following PRISMA guidelines. Articles were screened against pre-specified criteria to include lower-grade glioma (LGG, WHO Grade II/III) and glioblastoma (GBM, WHO Grade IV) separately. Progression free survival (PFS) and overall survival (OS) from Kaplan–Meier and multivariable analyses were outcomes of interest. Nine institutional studies describing 2193 IDH-mutant gliomas satisfied criteria for evaluation, with 1756 (80%) LGG and 437 (20%) GBM. When reported, the proportion of CDKN2A homozygous deleted gliomas ranged from 9 to 43%, with a median incidence of 22%. For LGG, Kaplan–Meier analyses demonstrated shorter PFS in the presence of CDKN2A homozygous deletion in three studies (median values, 31 versus 91 months), and shorter OS in five studies (median values, 61 versus 154 months). For GBM, Kaplan–Meier analyses demonstrated shorter PFS in the presence of CDKN2A homozygous deletion in two studies (median values, 16 versus 30 months), and shorter OS in four studies (median values, 38 versus 86 months). By multivariable analyses, CDKN2A homozygous deletion was a predictor of significantly shorter PFS and OS in both LGG and GBM across all included studies. The CDKN2A homozygous deletion is an important prognostic factor for survival outcomes of IDH-mutant glioma patients across multiple histologic WHO grades with specific molecular features likely dependent on IDH-mutant status. Greater understanding of how identifying this deletion can assist in the stratification of management for these tumors to optimize clinical course is required.

STUDY DESIGN Review. OBJECTIVE To provide practicing spine surgeons a primer with key insights for reading, interpreting, and clinically integrating systematic reviews and meta-analyses. SUMMARY OF BACKGROUND DATA Evidence-based medicine (EBM) refers to a family of standardized techniques for critical appraisal of clinical research. Within the contemporary spine literature, EBM is most commonly encountered in the form of systematic reviews and meta-analyses. Although these analytic techniques are potentially useful when appropriately applied to well-formulated questions with adequate primary data in the literature, the rapid and somewhat indiscriminate increase in volume of systematic reviews and meta-analyses published may be associated with an overall dilution in their quality, as well as misperceptions regarding the applicability of particular EBM studies to spine surgery in general. METHODS The collective experience of spine specialists with vested interests in advancing EBM and its utility was summarized into a primer. RESULTS We emphasize components that are pertinent methodologically (search strategy, study number, meta-analysis, bias, and certainty), and pertinent clinically (outcomes) to interpreting, and clinically integrating systematic reviews and meta-analyses into spine surgery practice. CONCLUSIONS Armed with these insights into these five perspectives, we anticipate that practicing spine surgeons will be better equipped to interpret systematic reviews and meta-analyses in a fashion that will meaningfully impact their patient care. LEVEL OF EVIDENCE 2.

Individual evidence suggests that multiple modalities can be used to treat entrapment pathology by Morton’s neuroma, including injection, neurolysis, and neurectomy. However, their impacts on patient pain and satisfaction have yet to be fully defined or elucidated. Correspondingly, our aim was to pool systematically identified metadata and substantiate the impact of these different modalities in treating Morton’s neuroma with respect to these outcomes. Searches of 7 electronic databases from inception to October 2019 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. The incidences of outcomes were extracted and pooled by random-effects meta-analysis of proportions. A total of 35 articles satisfied all criteria, reporting a total of 2998 patients with Morton’s neuroma managed by one of the three modalities. Incidence of complete pain relief after injection (43%; 95% CI, 23–64%) was significantly lower than neurolysis (68%; 95% CI, 51–84%) and neurectomy (74%; 95% CI, 66–82%) (P = 0.02). Incidence of complete satisfaction after injection (35%; 95% CI, 21–50%) was significantly lower than neurolysis (63%; 95% CI, 50–74%) and neurectomy (57%; 95% CI, 47–67%) (P

BACKGROUND Malignant peripheral nerve sheath tumors (MPNST) are rare tumors found throughout the body, with their clinical course in children still to be completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this demographic from a large national database. METHODS All MPNST patients aged ≤18 year in the U.S. National Cancer Database (NCDB) between 2005-2016 were retrospectively reviewed. Data were summarized and overall survival (OS) was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 251 pediatric MPNST cases were identified. Overall, mean age at diagnosis was 13.1 years (range, 1-18) with there being 132 (53%) females and 119 (47%) males. There were 84 (33%) MPNSTs located in extremities, 127 (51%) less than 1cm in size, and 22 (9%) have metastasis at diagnosis. In terms of treatment, surgery was pursued in 187 (74%) patients, chemotherapy in 116 (46%) patients, and radiation therapy in 129 (61%) patients. Five-year overall survival was estimated to be 52% (95% CI, 45-59%), and with median survival of 64 months (range, 36-136). Multivariate regression revealed older age (HR 1.10, P< 0.01), with metastases at time of diagnosis (HR 2.14, P=0.01), and biopsy only (HR 2.98, P< 0.01) all significantly and independently predicted shorter overall survival. Chemotherapy and radiation therapy were not statistically significant. CONCLUSIONS Pediatric MPNSTs are rare malignant entities, and only approximately half of patients will survive to five years. There remains a clear survival benefit in pursuing maximal safe resection in these patients. As such, judicious workup with meticulous surgical resection by an expert team should then be considered the standard of care for these tumors in children.

ImportanceHigh-grade gliomas (HGG) are the most aggressive and common malignant brain tumors in adults. They have a dismally fatal prognosis. Even if gross total resection of the enhancing tumor is achieved, inevitably, invading tumor cells that are indistinguishable to the un-aided eye are left behind, which eventually leads to tumor recurrence. 5-aminolevulinic acid (5-ALA) is an increasingly utilized intraoperative fluorescent imaging agent for patients with HGG. It enhances visualization of HGG tissue. Despite early promising randomized clinical trial data suggesting a survival benefit for 5-ALA-guided surgery, the growing body of literature must be analyzed to confirm efficacy on patient outcomes.Objective To perform a systematic review of the literature to evaluate whether there is a beneficial effect upon survival and extent of resection due to the utilization of 5-ALA in HGG surgery. Evidence ReviewLiterature regarding 5-ALA usage in HGG surgery was reviewed according to the PRISMA guidelines. One database, PubMed, was searched for assorted combinations of the keywords “5-ALA,” “high-grade glioma,” “5-aminolevulinic acid,” and “resection” in July 2020 for case reports and retrospective, prospective, and randomized clinical trials assessing and analyzing 5-ALA intraoperative use in patients with HGG. Entailed studies on PubMed and SCOPUS were found for screening using a snowball search technique upon the initially searched PubMed papers. Systematic reviews and meta-analyses were excluded from our PRISMA table. Findings1,951 previously published studies were screened, 536 of which were further evaluated, and ultimately 45 were included in our systematic review. There were no date restrictions on the screened publications. Our literature search was finalized on July 16, 2020. We found an observed increase in the overall survival (OS) and progression-free survival (PFS) of the 5-ALA group compared to the white light group, as well as an observed increase in the OS and PFS of complete resections compared to incomplete resections. Of the studies that directly compared the use of 5-ALA to white light (13 of the total analyzed 45, or 28.9%), 5-ALA lead to a better PFS and OS in 88.4% and 67.5% of patients, respectively. When the studies that reported postoperative neurologic outcomes of surgeries using 5-ALA vs. white light were analyzed, 42.2% of subjects demonstrated 5-ALA use was associated with less post-op neurological deficits, whereas 34.5% demonstrated no statistically significant difference between 5-ALA and without. 23.3% of studies showed that intraoperative 5-ALA guided surgeries lead to more post-op neurological deficits. Conclusions and RelevanceUtilization of 5-ALA was found to be associated with a greater extent of resection in HGG surgeries, as well as longer OS and PFS. Postop neurologic deficit rates were mixed and inconclusive when comparing 5-ALA groups to white light groups. 5-ALA is a useful surgical adjunct for resection of HGG when patient safety is preserved.

Surgical resection of high-grade brainstem gliomas is challenging and treatment mostly involves radiation and chemotherapy. In this study, we utilized registry data to determine prognostic features and impact of chemotherapy and radiation on overall survival. The National Cancer Database was queried from 2006 to 2015 for adult cases with histologically confirmed high-grade brainstem glioma. Covariates including patient demographics, comorbidities, tumor characteristics and treatment parameters were captured. Multivariable Cox proportional hazards regression was performed to identify predictors of survival. A total of 422 patients were analyzed. Most patients (66.6%) underwent postoperative radiation with chemotherapy, 9.2% underwent radiation alone, while the remaining had no postoperative treatment (24.2%). Overall median survival was 9.8 months (95% CI 8.8–12). Survival was longer (p

Post-traumatic hydrocephalus (PTH) is a potentially morbid sequela of decompressive craniectomy for traumatic brain injury (TBI). Subdural hygromas are commonly identified following decompressive craniectomy, but the clinical relevance and predictive relationship with PTH in this patient cohort is not completely understood. Survey of seven electronic databases from inception to June 2019 was conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. Multivariate hazard ratios (HRs) for PTH by the presence of subdural hygroma were extracted and pooled by meta-analysis of proportions with random effects modeling. We systematically identified nine pertinent studies describing outcomes of 1010 TBI patients managed by decompressive craniectomy. Of the overall cohort, there were 211 (21%) females and median age was 37.5 years (range 33–53). On presentation, median Glasgow Coma Scale was 7 (range, 5–8). In sum, PTH was reported in 228/840 (27%) cases, and subdural hygroma was reported in 449/1010 (44%) cases across all studies. Pooling multivariate-derived HRs indicated that subdural hygroma was a significant, independent predictor of PTH (HR, 7.1; 95% CI, 3.3–15.1). The certainty of this association was deemed low due to heterogeneity concerns. The presence of subdural hygroma is associated with increased risk of PTH after decompressive craniectomy among TBI patients based on the current literature and may mandate closer clinical surveillance when detected. Prospective studies, including those of intracranial hydrodynamics following decompressive craniectomy in the setting of TBI, will better validate the certainty of these findings.

Background Aneurysm rerupture and bilateral fixed and dilated pupils (bFDPs) typically have a poor prognosis across all age groups. The synchronous occurrence of both features in the infantile demographic has not been previously reported. Case Description We describe the near-fatal clinical course of a 7-month-old infant who experienced an acute giant aneurysm rerupture with signs of bFDPs. The patient was rapidly managed with neurosurgical intervention and has achieved a favorable outcome 1 year later. Conclusions This case highlights that survival of infants who present with intracranial aneurysmal rerupture and bFDPs is amenable to rapid neurosurgical intervention and should be remembered in clinical practice.

Multiple studies have reported the loss of trimethylation at lysine (K) 27 on histone 3 (H3K27me3) in high-grade malignant peripheral nerve sheath tumors (MPNSTs). However, the diagnostic potential of this finding in MPNSTs remains yet to be fully substantiated. Correspondingly, our aim was to pool systematically-identified metadata in the literature and substantiate the incidence of H3K27me3 loss in this setting. Searches of 7 electronic databases from inception to May 2019 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. The incidence of loss was then pooled by random-effects meta-analysis of proportions. Nine pertinent studies described a total of 823 high-grade MPNST samples. When pooled, incidence (sensitivity) of complete H3K27me3 loss was estimated to be 53% (95% CI 42–64%). For MPNST subtypes, estimated incidences of complete loss in NF1 subtype was 52% (95% CI 41–62), in sporadic subtype was 53% (95% CI 36–70%), in the epithelioid subtype was 0% (95% CI 0–7%), and radiation-associated subtype was 98% (95% CI 86–100%). Finally, incidence of incomplete loss (specificity) in 1231 MPNST-mimic samples was estimated to be 96% (95% CI 90–99%). Certainty of these outcomes ranged from very low to high. The incidence of complete H3K27me3 loss is substantial in high-grade MPNSTs and is low in MPNST-mimics. Greater cohort study and biological investigation will validate the certainty of these findings as well as elucidate their true molecular and clinical significances.

Objective Traditionally, extent of resection (EOR) has been seen as a surgical parameter that can predict survival outcomes of surgically managed WHO grade II gliomas. The aim of this study was to evaluate if such an influence was potentially affected by 1p/19q codeletion status based on a national cancer registry. Patient and methods All adults diagnosed with grade II gliomas between the years 2004 to 2014 were queried from the National Cancer Database (NCDB). The population was then divided based on 1p/19q codeletion status, and then Kaplan-Meier, univariate and multivariate Cox regression analyses were utilized to evaluate the prognostic effect of EOR. Results In total, 1,498 grade II gliomas satisfied inclusion for analysis, with the 1p/19q non-codeleted in 705 (47%) cases, and codeletion in 793 (53%) cases. When the cohort was divided based on codeletion status, Kaplan-Meier modelling and univariate regression analyses indicated that gross total resection (GTR) was significantly associated with greater 5-overall survival (OS) in both 1p/19q non-codeleted and codeletion groups. Upon multivariate analysis which incorporated adjuvant therapy status, the significance of GTR was only retained in the 1p/19q non-codeletion group after post-hoc adjustment. Conclusion Our findings indicate that the survival impact of GTR in grade II gliomas may be affected by 1p/19q codeletion status within the first five years after surgery based on overall survival. Therefore, molecular diagnostics have potential clinical application in surgery outcomes, and validation of the reported findings will assist in surgical planning if such an association can be thoroughly established.

Although the dismal clinical prognosis of diffuse intrinsic pontine glioma (DIPG) has not changed, there has been significant progress in the academic literature made in the biological understanding of this brainstem tumor. The aim of this analysis was to evaluate citation and other bibliometric characteristics of the 100 most-cited DIPG articles in the current literature in order to better understand the current state of our academic efforts in this area. Elsevier’s Scopus database was searched for the 100 most-cited articles that focussed on DIPG. Articles were dichotomized as either primarily basic science (BSc) or clinical (CL) articles. Various bibliometric parameters were summarized and comparison between BSc and CL articles was performed using Pearson’s chi-square and Mann–Whitney U tests. Of the 100 most-cited articles, 36 (36%) were BSc and 64 (64%) were CL articles. Overall median values were as follows: citation count, 52 (range, 27–261); citation rate per year, 8.6 (range, 1.7–104); number of authors, 9 (range, 1–63); and publication year, 2011 (range, 1997–2017). Articles were published in a total of 43 different journals and predominately originated in the USA (n = 67, 67%). When compared with CL articles, BSc articles reported significantly greater citation count (P = 0.03), citations rate per year (P

Idarucizumab is the first Food and Drugs Administration (FDA) approved reversal agent for anticoagulant dabigatran, a direct thrombin inhibitor. Emerging evidence suggests idarucizumab can improve clinical outcome following dabigatran-associated hemorrhage, however, its specific use in intracranial hemorrhage has been poorly described. The aim of this study was to systematically review the available literature of idarucizumab in the setting of dabigatran-associated ICH to evaluate its efficacy in the stabilizing/resolving of the primary hemorrhage. A systematic search of 7 electronic databases from their earliest records to August 2018 was conducted following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. There were 864 articles identified for screening against selection criteria. The search identified 9 articles to be included in our analysis, describing hemorrhage outcomes in 23 dabigatran-associated cases of ICH managed by idarucizumab. Mean overall age was 76.2 years, with 43% females, and bleeding was subdural, subarachnoid and intracerebral in 43%, 13% and 43% cases respectively. Surgical intervention was pursued in 48% of cases. During the course of the hospitalization, the hemorrhages stabilized/resolved in 87% of patients, and worsened in 13%. In-hospital complications occurred in 4% of cases, and mortality occurred in 4% of cases as well. The available literature suggests that idarucizumab can be applied in the setting of ICH, for its therapeutic effect in patients presenting with dabigatran-associated ICH appears acceptable with no compromise to clinical safety. However, currently there is a paucity of data about various aspects that are involved in other aspects of ICH treatment, including recovery, that limits the significance of the current literature. As more evidence is published relating specifically to long-term ICH outcomes that have been treated by idarucizumab, we will be better placed to establish the optimal role of idarucizumab in the setting of dabigatran-associated ICH.

Rathke's cleft cysts (RCCs) are benign growths of the embryological Rathke's pouch. Surgical decompression provides effective symptomatic relief in most cases; however, the effect of gross total resection (GTR) of the cyst wall on recurrence, as well as pituitary function, is unclear. The aim of this meta-analysis was to pool the current literature and ascertain the recurrence control afforded by GTR of the cyst wall compared with subtotal resection (STR). Searches of seven electronic databases from inception to January 2019 were conducted following PRISMA guidelines, resulting in 476 articles to be screened. Outcomes were analyzed using meta-analysis of proportions. A total of 10 retrospective cohort studies satisfied selection criteria, describing 655 surgically managed RCC cases, with 254 (39%) and 401 (61%) achieving GTR and STR of the cyst wall, respectively. GTR was associated with significantly reduced overall RCC recurrence by fixed-effects (FE) modeling (RR, 0.66; 95% CI, 0.45-0.96), but not by random effects (RE) modeling (RR, 0.75; 95% CI, 0.51-1.12). Based on both models, GTR was associated with significantly reduced symptomatic recurrence (RE model, RR, 0.37, 95% CI, 0.14-0.95) and significantly increased postoperative diabetes insipidus (RE model, RR, 2.60; 95% CI, 1.34-5.03). There was insufficient data to evaluate other pituitary axes in this context. The current evidence indicates that GTR of the RCC cyst wall has the potential to affect the incidence of overall and symptomatic RCC recurrences, as well as drive postoperative DI incidence. However, expectations of clinical and pragmatic benefit following cyst wall resection should be titrated carefully against the potential for postoperative and pituitary morbidities which currently remain poorly defined. Greater granularity is required to understand all factors that can influence recurrence and quality of life when evaluating resection of RCC.

Subtotal resection (STR) of vestibular schwannoma (VS) tumors remains controversial and little is known regarding post-operative volume changes. Authors retrospective reviewed the medical records from January 1st 2002 to January 1st 2018, for all patients who had undergone primary STR of large VS at a single tertiary academic institution. Our series consists of 34 patients with a mean age of 53.9 (median 53; range 21–87) years that had STR of their VS tumor. The mean pre-operative tumor diameter and volume was 3.9 cm (median 3.0 cm; range 1.6–6.0 cm) and 11.7 cm3 (median 9.6 cm3; range 2.8–44.3 cm3), respectively, with a mean extent of resection of 86% (median 90%; range 53–99%). The mean radiographic and clinical follow-up was 40 months (range 6–120 months) and 51 months (range 7–141 months), respectively. 85% of patients had optimal House–Brackmann (HB) scores (Grade 1 & 2) immediately post-operatively, and 91% at 1 year; 94% of patients had normal (HB 1) at last follow-up. There was significant regression of residual tumor volume at 1 year (p = 0.006) and 2 years (p = 0.02), but not at 3 years (p = 0.08), when compared to the prior year. There was significant regression of size over time, with a mean slope estimate of − 0.70 units per year (p

Systematic review and meta-analysis of randomised controlled trials. The purpose of this study is to perform a systematic review and meta-analysis of antifibrinolytic agents for paediatric spine surgery. Bleeding is an important consideration in paediatric scoliosis surgery; blood loss leads directly to higher morbidity and mortality. Antifibrinolytics are an attractive non-invasive method of reducing bleeding as evidenced in arthroplasty, cardiac surgery and adult scoliosis surgery. A thorough database search of Medline, PubMed, EMBASE and Cochrane was performed according to PRISMA guidelines, and a systematic review was performed. Five randomised controlled trials were identified in this meta-analysis, consisting of a total of 285 spine surgery patients with subgroups of tranexamic acid (n = 101), epsilon aminocaproic acid (n = 61) and control (n = 123). This meta-analysis found that antifibrinolytics lead to statistically significant reductions in peri-operative blood loss (MD − 379.16, 95% CI [− 579.76, − 178.57], p

The resection of insular gliomas remains a neurosurgical challenge due to the close proximity of functionally-important cortical, white matter tracts, and vasculature structures. More recently, the feasibility of resection has gained traction, however, there is a lack of consolidated neurological deficit metrics. Thus, the aim of this study was to determine the incidences of neurological deficits following insular glioma resection to better guide selection algorithms and resource allocations. Searches of seven electronic databases from inception to August 2018 were conducted following Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Data were extracted and pooled using meta-analysis of proportions. Meta-regression was used to identify potential sources of heterogeneity. Nineteen observational studies reported the neurological outcomes of 890 insular glioma patients. The pooled incidences of new temporary and permanent motor deficits were 11% (95% CI, 6-17%) and 4% (95% CI, 2-7%) respectively, and new temporary and permanent language deficits were 11% (95% CI, 6-17%) and 2% (95% CI, 0-4%) respectively. Single-surgeon series reported significantly lower incidences of both permanent motor (2% vs 7%; P 0.001) and language (1% vs 3%; P = 0.03) deficits. The incidences of motor and language neurological deficits following insular glioma resection have been quantified, and will assist in determining the suitability and appropriateness of pursuing surgical resection for insular glioma. We note that permanent neurological deficits are lowest when reported by series describing outcomes of a single surgeon, indicating most optimal outcomes may be best achieved after intense training and/or greater experience.

The incidence of extraosseous Ewing’s sarcoma, a highly malignant mesenchymal mass, is rare in the spinal cord and its clinical outcomes unknown. To date, few cases of primary intradural extramedullary Ewing’s sarcoma (PIEES) have been reported, with varying follow-up. Herein, we aimed to perform a comprehensive review of all cases published in the literature and update previously reported cases from our institution. Institutional medical records were searched for cases of PIEES of the spine managed at our institution between the years 2005 and 2018. We performed a systematic search of two electronic databases (Ovid Medline and Embase) from inception to August 2018 to obtain all published cases of primary intradural extraosseous Ewing’s sarcoma (PIEES).We used our institutional medical records to update cases reported from our institution. We identified a total of 44 cases with pIEES reported in the existing literature of which 5 have been from our department. Of the 41 cases, the median age of diagnosis was 31 years. The most common presentation pattern was PIEES in the lumbar/sacral region (61%, n = 27), with a majority (59%, n = 25) presenting initially with pain. The most common modality of treatment reported was surgery (41/41, 100%), followed by adjuvant chemotherapy (31/44, 70%) and local radiation therapy (29/44, 66%). Overall, recurrence was reported in 17/36 (46%) cases, with median progression free survival (PFS) of 12 months (range, 1–72). There were 12/37 (32.4%) deaths reported, with median overall survival (OS) of 14 months (range, 1–72). Hence, we present the most updated review of all reported cases of PIEES. While surgical resection is the mainstay of treatment, tumor recurrence is a great concern given the adhesive nature of the lesion preventing complete resection. Adjuvant chemotherapy and radiotherapy should be carefully considered to prevent recurrence and improve survival outcome.

OBJECTIVE Lumbar stenosis treatment has evolved with the introduction of minimally invasive surgery (MIS) techniques. Endoscopic methods take the concepts applied to MIS a step further, with multiple studies showing that endoscopic techniques have outcomes that are similar to those of more traditional approaches. The aim of this study was to perform an updated meta-analysis and systematic review of studies comparing the outcomes between endoscopic (uni- and biportal) and microscopic techniques for the treatment of lumbar stenosis. METHODS Following PRISMA guidelines, a systematic search was performed using the Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Ovid Embase, and PubMed databases from their dates of inception to December 14, 2020. All identified articles were then systematically screened against the following inclusion criteria: 1) studies comparing endoscopic (either uniportal or biportal) with minimally invasive approaches, 2) patient age ≥ 18 years, and 3) diagnosis of lumbar spinal stenosis. Bias was assessed using quality assessment criteria and funnel plots. Meta-analysis using a random-effects model was used to synthesize the metadata. RESULTS From a total of 470 studies, 14 underwent full-text assessment. Of these 14 studies, 13 comparative studies were included for quantitative analysis, totaling 1406 procedures satisfying all criteria for selection. Regarding postoperative back pain, 9 studies showed that endoscopic methods resulted in significantly lower pain scores compared with MIS (mean difference [MD] −1.0, 95% CI −1.6 to −0.4, p < 0.01). The length of stay data were reported by 7 studies, with endoscopic methods associated with a significantly shorter length of stay versus the MIS technique (MD −2.1 days, 95% CI −2.7 to −1.4, p < 0.01). There was no significant difference with respect to leg visual analog scale scores, Oswestry Disability Index scores, blood loss, surgical time, and complications, and there were not any significant quality or bias concerns. CONCLUSIONS Both endoscopic and MIS techniques are safe and effective methods for treating patients with symptomatic lumbar stenosis. Patients who undergo endoscopic surgery seem to report less postoperative low-back pain and significantly reduced hospital stay with a trend toward less perioperative blood loss. Future large prospective randomized trials are needed to confirm the findings in this study.

Background Given the growing prevalence of initial endovascular treatment for type 1 spinal dural arteriovenous fistulae (dAVF), there are an increasing number of patients presenting with progressive symptoms related to recurrent previously embolized spinal dAVF. This study's goal was to identify demographic, clinical, and radiographic variables among patients who have failed embolization of type I spinal dAVF. Methods A retrospective review of 24 consecutive surgeries for type I spinal dAVF performed by the senior author (A.D.L.) identified 5 patients who underwent open surgery for failed embolization. These 5 cases were reviewed for location of fistula, time from embolization to recurrence, preoperative functional status, fistulous point encountered at surgery, and clinical outcome of the patient at 3-month follow-up. A representative example case is reviewed in detail. Results The median age at time of recurrence was 63 years (range 51–73 years). The median timing of embolization to recurrence of neurologic symptoms was 5 months (range 1–54) and to surgery 7 months (range 2–60 months). The level of the spinal dAVF was most frequently at T12–L1 (n = 3). Spinal magnetic resonance arteriography led to localization of the spinal dAVF in 2 patients and spinal catheter angiogram in 3 cases. All patients had definitive radiographic cure of the dAVF at last clinical follow-up. Conclusions The increased use of endovascular treatment of spinal dAVF has led to the treatment of refractory cases with a greater degree of surgical complexity. Open surgical ligation continues to provide the most definitive treatment outcomes for this complex spinal vascular entity.

Background Adult craniopharyngiomas are low-grade tumors of the pituitary infundibulum that can be locally aggressive and frequently present with profound visual deficits and endocrinopathies. Surgical resection remains the preferred initial treatment for these lesions, and recently endoscopic endonasal approaches (EEAs) have become increasingly used. However, minimal data exist comparing these techniques with traditional transcranial (TC) methods. The purpose of this study was to evaluate perioperative differences in EEA and TC approaches for adult craniopharyngiomas over the past several decades. Methods Craniopharyngioma surgeries in the Nationwide Inpatient Sample from 1998 to 2014 were identified. Complication rates, mortality rates, and annual treatment trends were stratified by procedure. Annual caseload was assessed with linear regression, and multivariate logistic regression models were created to determine predictors of inpatient mortality and perioperative complications. Results From 1998−2014, a significant increase in EEAs for craniopharyngiomas (+4.36/year, r2 = 0.80, P Conclusions Over the past several decades, utilization of EEAs to resect adult craniopharyngiomas has increased. EEAs appear to be associated with lower rates of perioperative mortality and complications. However, long-term, prospective studies controlling for tumor size, location, and preoperative symptomatology are needed to determine when one approach should be used preferentially over the other.

Background Laser interstitial thermal therapy (LITT) for posterior fossa lesions remains rare as the small size of the infratentorial compartment, proximity to the brainstem, and thickness/angulation of the occipital bone creates barriers to procedural success. Furthermore, evaluation of the effect of ablation volume on outcomes is limited. Objective To analyze our institutional experience with LITT in the posterior fossa stratifying perioperative and long-term outcomes by ablation volumes. Methods Seventeen patients with posterior fossa lesions treated with LITT from 2013 to 2020 were identified. Local progression-free survival (PFS), overall survival, steroid dependence, and edema reduction were evaluated with Kaplan-Meier analysis grouped by ablation volume. Preoperative, postoperative, and last known Karnofsky Performance Status (KPS) were compared using a matched paired t test. Results No differences in pathology, preoperative KPS, or preoperative lesion volume were found between patients with total (100%-200% increase in pre-LITT lesion volume) versus radical (>200% increase in pre-LITT lesion volume) ablations. Patients who underwent radical ablation had a higher postoperative KPS (93 vs 82, P = .02) and higher KPS (94 vs 87, P = .04) and greater reduction in perilesional edema at last follow-up (P = .01). Median follow-up was 80.8 wk. Conclusion Despite obvious anatomical challenges, our results demonstrate that radical ablations are both feasible and safe in the posterior fossa. Furthermore, radical ablations may lead to greater decreases in perilesional edema and improved functional status both immediately after surgery and at last follow-up. Thus, LITT should be considered for patients with otherwise unresectable or radioresistant posterior fossa lesions.

Frozen section is a time- and labor-intensive method for intraoperative pathologic diagnosis. As a result, there exists a need to expedite and streamline the acquisition and interpretation of diagnostic histologic data to inform surgical decision making. Stimulated Raman histology (SRH) is an emerging technology that may serve to expedite the acquisition and interpretation of histologic data in the operating room.A blinded, prospective cohort study of 82 patients undergoing resection for tumors of the central nervous system was performed. Twenty-six patients with diagnoses of meningioma on SRH, frozen, or permanent section were included in this subanalysis. Diagnostic time and accuracy of stimulated SRH histology images were compared with the gold standard (frozen section). Agreement of SRH and frozen section diagnosis with permanent section (true) diagnosis was also compared.Mean time-to-diagnosis was significantly shorter for SRH-mediated diagnosis compared with frozen section (9.2 vs. 35.8, P0.0001). Diagnostic accuracy was not significantly different between methods (P = 0.15). Diagnostic agreement was not significantly different between SRH versus frozen, SRH versus permanent, or frozen versus permanent section methods (P = 0.5, P = 0.5, P = 1.00).SRH is a promising adjuvant technology that may expedite intraoperative neuropathologic consult without sacrificing diagnostic accuracy.

OBJECTIVE Cerebral extracranial-intracranial (EC-IC) direct bypass is a commonly used procedure for ischemic vasculopathy. A previously described variation of this technique is to utilize one donor artery to supply two recipient arteries, which the authors designate as 1D2R. The purpose of this study is to present a single surgeon’s series of 1D2R direct bypasses for moyamoya and ischemia using detailed clinical, angiographic, and intraoperative blood flow measurement data. To the authors’ knowledge, this is the largest series reported to date. METHODS Hospital, office, and radiographic imaging records for all patients who underwent cerebral revascularization using a 1D2R bypass by the senior author were reviewed. The patients’ demographic information, clinical presentation, associated medical conditions, intraoperative information, and postoperative course were obtained from reviewing the medical records. RESULTS A total of 21 1D2R bypasses were performed in 19 patients during the study period. Immediate bypass patency was 100% and was 90% on delayed follow-up. The mean initial cut flow index (CFI(i)) was 0.64 ± 0.33 prior to the second anastomosis and the mean final value (CFI(f)) was 0.94 ± 0.38 after the second anastomosis (p < 0.001). The overall bypass flow increased on average by 50% (mean 17.9 ml/min, range −10 to 40 ml/min) with the addition of the second anastomosis. There was no significant difference in the overall flow measurements when the end-to-side anastomosis or side-to-side anastomosis was performed first. There was a statistically significant difference in the proportion of patients with a modified Rankin Scale (mRS) score of 0 or 1 postoperatively compared to preoperatively (p < 0.01). Through the application of Poiseuille’s law, the authors analyzed flow dynamics, deduced the component vascular resistances based on an analogy to electrical circuits and Ohm’s law, and introduced the new concepts of “second anastomosis relative augmentation” and “second anastomosis sink index” in the evaluation of 1D2R bypasses. CONCLUSIONS The application of the 1D2R technique in a series of 19 consecutive patients undergoing direct EC-IC bypass for flow augmentation demonstrated high patency rates, statistically significantly higher CFIs compared to 1D1R, and improved mRS scores at last clinical follow-up. Additionally, the technique allows a shorter dissection time and preserves blood flow to the scalp. The routine utilization of intraoperative volumetric flow measurements in such surgeries allows a deeper understanding of the hemodynamic impact on individual patients.

Background Successful management of pediatric low-grade glioma (pLGG) can be complicated by eloquent anatomical location, as well as specific pathologic and molecular features. Some authors have proposed using the VEGF inhibitor bevacizumab to improve disease control, but its safety and efficacy are poorly defined. Correspondingly, our aim was to pool systematically identified clinical data in the literature to assess the clinical utility of bevacizumab for pLGG at progression. Methods A systematic search of 7 electronic databases from inception to June 2019 was conducted following PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. Articles were screened against prespecified criteria. Outcomes were then pooled by random-effects meta-analyses of proportions. Results Seven pertinent studies described the outcomes of 110 progressive pLGG patients managed with bevacizumab in largely multiagent regimens. While on treatment, the rate of clinical response was 58% (95% CI, 43%-72%), and the rate of response on imaging was 80% (95% CI, 58%-96%). The rate of grade 3 or higher toxicity was 8% (95% CI, 2%-17%), with proteinuria the most commonly described. In the off-treatment period up to median 1 year, the rate of progression was estimated to be 51% (95% CI, 28%-74%). Conclusions Bevacizumab has the potential to control clinical and radiographic disease with relatively low grade 3 or higher toxicity risk in progressive pLGG patients. However, the long-term off-treatment benefits of this therapy are not yet well defined. Heterogeneity in the literature precludes any formal recommendations regarding its use until larger, more standardized investigations can be performed.

OBJECTIVE Syringomyelia is a debilitating, progressive disease process that can lead to loss of neurological function in patients already experiencing significant compromise. Syringosubarachnoid, syringoperitoneal, and syringopleural shunts are accepted treatment options for patients with persistent syringomyelia, but direct comparisons have been lacking to date. The authors conducted a systematic review of the literature and meta-analysis to compare clinical outcomes between these three syrinx shunt modalities. METHODS Utilizing PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for systematic reviews, Ovid Embase, PubMed, Scopus, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, American College of Physicians Journal Club, and Database of Abstracts of Review of Effectiveness were searched to identify all potentially relevant studies published from inception until July 2020. Data were extracted and analyzed using meta-analysis of proportions. The primary study outcome was the rate of reoperation based on the initial shunt modality. Secondary outcomes included clinical improvement, clinical deterioration, and complications following shunt placement. RESULTS A total of 22 articles describing 27 distinct treatment cohorts published between 1984 and 2019 satisfied the inclusion criteria. This captured 473 syrinx shunt procedures, 193 (41%) by syringosubarachnoid shunt, 153 (32%) by syringoperitoneal shunt, and 127 (27%) by syringopleural shunt, with an overall median clinical follow-up of 44 months. The pooled incidences of revision surgery were estimated as 13% for syringosubarachnoid, 28% for syringoperitoneal, and 10% for syringopleural shunts, respectively (p-interaction = 0.27). The rate of clinical improvement was estimated as 61% for syringosubarachnoid, 64% for syringoperitoneal, and 71% for syringopleural shunts. The rate of clinical deterioration following placement was estimated as 13% for syringosubarachnoid, 13% for syringoperitoneal, and 10% for syringopleural shunts. CONCLUSIONS The preferred modality of syrinx shunting remains a controversial topic for symptomatic syringomyelia. This study suggests that while all three modalities offer similar rates of clinical improvement and deterioration after placement, syringoperitoneal shunts have a greater rate of malfunction requiring surgical revision. These data also suggest that syringopleural shunts may offer the best rate of clinical improvement with the lowest rate of reoperation.

Background The intracerebral occurrence of malignant peripheral nerve sheath tumors (MPNSTs) is exceedingly rare, and despite aggressive treatments, local recurrence and poor prognosis are very frequent. Like other brain tumors, these tumors could be primary or secondary, making the term "peripheral" an imprecise term for a primary brain tumor. Objective To analyze the reported cases of primary and secondary cerebral MPSNTs in terms of diagnosis, treatment, and overall survival. Additionally, we present a case of malignant intracerebral nerve sheath tumor (MINST) treated with radical surgery and radiotherapy. Methods Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, one database (PubMed) and crossed references were queried for MPNST with brain metastasis and primary MINSTs from 1971 to 2020. Data regarding demographic features, primary tumor site, risk factors, brain location of the lesion, treatment applied, and overall survival were extracted. Results A total of 55 patients were selected (including the reported case): 29 patients were secondary brain MPNST and 26 patients were primary MINST. The mean age was 41.8 ± 22 and 31.2 ± 23 yr, respectively. All brain metastases of MPNST (100%) had a primary tumor elsewhere in the body at the time of diagnosis. The overall survival was significantly shorter in patients with a secondary brain MPNST compared to MINST (P = .002). Conclusion We present a comprehensive analysis of every reported primary and secondary intracerebral MPNST. The prognosis in terms of survival is worst in the last one despite aggressive treatment. The lack of a primary MPNST in screening tests is sufficient to confirm a MINST at time of diagnosis.

Background Treatment for glioblastoma (GBM) in elderly (age > 65 years) patients can be affected by multiple geographic and socioeconomic parameters. Correspondingly, the aim of this study was to determine trends in treatment of elderly GBM patients in the United States. Methods All GBM patients in the U.S. National Cancer Database between 2005 and 2016 were retrospectively reviewed. Status of treatment by triple therapy (resection, chemotherapy, and radiation) were summarized and analyzed by U.S. Census region. Results There were 44 338 GBM patients included, with 21 573 (49%) elderly and 22 765 (51%) nonelderly patients with median ages 72 years (range, 65-90 years) and 47 years (range, 40-64 years), respectively. Compared to nonelderly patients, elderly patients had significantly lower odds of being treated by triple therapy (odds ratio, OR = 0.54) as a whole, and its individual elements of resection (OR = 0.78), chemotherapy (OR = 0.46), radiation therapy (OR = 0.52). This was reflected in each U.S. Census region, with the lowest odds of being treated with triple therapy, surgical resection, chemotherapy, and radiation therapy in New England (OR = 0.51) Mountain (OR = 0.66), West North Central (OR = 0.38), and the Middle Atlantic (OR = 0.44), respectively. Multivariable analysis revealed multiple socioeconomic parameters that significantly predicted lower odds of triple therapy in the elderly. Conclusions In the United States alone, there exists geographic disparity in the treatment outcomes of elderly GBM patients. Multiple socioeconomic parameters can influence access to treatment modalities for elderly patients compared to younger patients in different geographic regions, and public health initiatives targeting these aspects may prove beneficial conceptually to optimize and homogenize clinical outcomes.

OBJECTIVEAdult glioblastoma (GBM) has proven refractory to decades of innovation. Oncolytic viral therapy represents a novel therapy that uses viral vectors as both a delivery and therapeutic mechanism to target GBM cells. Despite the growing body of basic science data supporting the feasibility of viral therapy to treat GBM, the reporting of clinical trial results is heterogeneous. Correspondingly, the aim of this study was to present a contemporary summary of the progress all clinical trials have made to date.METHODSThe ClinicalTrials.gov database was reviewed in August 2020 for all possible interventional clinical trials involving viral vector–based therapy to treat adult GBM. These were then screened against selection criteria to identify pertinent clinical trials.RESULTSA total of 29 oncolytic viral therapy trials treating adult GBM were identified. The median start and expected completion years were 2014 and 2020, respectively. At the time of this writing, 10 (35%) trials were reported to have completed recruitment, whereas 7 (24%) were actively recruiting. The median target enrollment number was 36 (range 13–108), with the majority of trials being phase I (n = 18, 62%), and involving secondary GBM among other malignant glioma (n = 19, 66%). A total of 10 unique viral vectors were used across all trials, with the most common being adenovirus (n = 16, 55%). Only 2 (7%) phase I trials to date have reported outcomes on the ClinicalTrials.gov portal. Results of 12 additional clinical trials were found in academic publications, with median progression-free and overall survival times of 3 and 15 months, respectively, after the first viral dose at recurrence. The coordination of the large majority of trials originated from the US (n = 21, 72%), and the median number of testing sites per trial was 1 (range 1–15), via industry funding (n = 18 trials, 62%).CONCLUSIONSThere are multiple early-stage oncolytic viral therapy clinical trials for adult GBM currently active. To date, limited results and outcomes are promising but scarce. The authors expect this to change in the near future because many trials are scheduled to have either nearly or actually reached their expected recruitment completion time. How exactly oncolytic viral therapy will fit into the current treatment paradigms for primary and secondary GBM remains to be seen, and will not be known until safety and toxicity profiles are established by these clinical trials.

The prediction of outcome after mechanical thrombectomy (MT) of basilar artery occlusion (BAO) remains an area of investigation. The objective of this study was to evaluate the prognostic role of presenting National Institute Health of Stroke Scale (NIHSS) scores in predicting favorable 90-day functional outcome. A survey of 7 electronic databases from inception to May 2020 was conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. Multivariate odds ratios (ORs) for favorable 90-day function outcome (modified Rankin Score 0-2) were extracted and pooled by meta-analysis of proportions with random effects modeling. A total of 10 individual studies satisfied criteria for selection and described a total of 941 BAO patients managed by MT. Analysis revealed 590 (63%) males with a mean age of 66.6 years. The median presenting NIHSS was 19, and 316 (34%) patients were reported to have a favorable functional status 90-days after treatment. Lower presenting NIHSS scores independently and significantly predicted favorable 90-day functional outcome in BAO patients with a pooled OR of 0.89 (95% CI, 0.87-0.92; I2 = 18%; P-heterogeneity = 0.28). Meta-regression did not detect any clinical parameter that influenced this trend direction or its significance, and bias assessments were unremarkable. We confirm in this study via a consensus within the literature that the presenting NIHSS score predicts 90-day functional outcome in BAO patients treated by MT. Further, its standardized use allows more meaningful comparisons between interventions and anatomical locations.

BACKGROUND Evolving requirements for patient and physician safety and rapid regulatory changes have stimulated interest in neurosurgical telemedicine in the COVID-19 era. OBJECTIVE To conduct a systematic literature review investigating treatment of neurosurgical patients via telemedicine, and to evaluate barriers and challenges. Additionally, we review recent regulatory changes that affect telemedicine in neurosurgery, and our institution's initial experience. METHODS A systematic review was performed including all studies investigating success regarding treatment of neurosurgical patients via telemedicine. We reviewed our department's outpatient clinic billing records after telemedicine was implemented from 3/23/2020 to 4/6/2020 and reviewed modifier 95 inclusion to determine the number of face-to-face and telemedicine visits, as well as breakdown of weekly telemedicine clinic visits by subspecialty. RESULTS A total of 52 studies (25 prospective and 27 retrospective) with 45 801 patients were analyzed. A total of 13 studies were conducted in the United States and 39 in foreign countries. Patient management was successful via telemedicine in 99.6% of cases. Telemedicine visits failed in 162 cases, 81.5% of which were due to technology failure, and 18.5% of which were due to patients requiring further face-to-face evaluation or treatment. A total of 16 studies compared telemedicine encounters to alternative patient encounter mediums; telemedicine was equivalent or superior in 15 studies. From 3/23/2020 to 4/6/2020, our department had 122 telemedicine visits (65.9%) and 63 face-to-face visits (34.1%). About 94.3% of telemedicine visits were billed using face-to-face procedural codes. CONCLUSION Neurosurgical telemedicine encounters appear promising in resource-scarce times, such as during global pandemics.