Cavernous malformations of the central nervous system: An international consensus statement.

Introduction: Cavernous malformations (CM) of the central nervous system constitute rare vascular lesions. They are usually asymptomatic, which has allowed their management to become quite debatable. Even when they become symptomatic their optimal mode and timing of treatment remains controversial. Research question: A consensus may navigate neurosurgeons through the decision-making process of selecting the optimal treatment for asymptomatic and symptomatic CMs. Material and methods: A 17-item questionnaire was developed to address controversial issues in relation to aspects of the treatment, surgical planning, optimal surgical strategy for specific age groups, the role of stereotactic radiosurgery, as well as a follow-up pattern. Consequently, a three-stage Delphi process was ran through 19 invited experts with the goal of reaching a consensus. The agreement rate for reaching a consensus was set at 70%. Results: A consensus for surgical intervention was reached on the importance of the patient's age, symptomatology, and hemorrhagic recurrence; and the CM's location and size. The employment of advanced MRI techniques is considered of value for surgical planning. Observation for asymptomatic eloquent or deep-seated CMs represents the commonest practice among our panel. Surgical resection is considered when a deep-seated CM becomes symptomatic or after a second bleeding episode. Asymptomatic, image-proven hemorrhages constituted no indication for surgical resection for our panelists. Consensus was also reached on not resecting any developmental venous anomalies, and on resecting the associated hemosiderin rim only in epilepsy cases. Discussion and conclusion: Our Delphi consensus provides an expert common practice for specific controversial issues of CM patient management.

Length of Survival, Outcome, and Potential Predictors in Poor-Grade Aneurysmal Subarachnoid Hemorrhage Patients Treated with Microsurgical Clipping.

BACKGROUND: Poor-grade aneurysmal subarachnoid hemorrhage (aSAH) has been associated with severe morbidity and high mortality. It has been demonstrated that early intervention is of paramount importance. The aim of our study is to evaluate the functional outcome and the overall survival of early microsurgically treated patients. MATERIAL AND METHODS: Poor-grade aSAH patients admitted at our institution over fifteen years (January 2008 - December 2022) were included in our retrospective study. All participants underwent brain Computed Tomography Angiography (CTA). Fisher scale was used to assess the severity of hemorrhage. All our study participants underwent microsurgical clipping, and their functional outcome was assessed with the Glasgow Outcome Scale (GOS). We used logistic regression analysis to identify any parameters associated with a favorable outcome at 12 months. Cox proportional hazard analysis was also performed, identifying factors affecting the length of survival. RESULTS: Our study included 39 patients with a mean age of 54 years. Thirty of our participants (76.9%) were Hunt and Hess grade V, while the vast majority (94.9%) were Fisher grade 4. The observed six-month mortality rate was 48.6%. The mean follow-up time was 18.6 months. The functional outcome at six months was favorable in 6 patients (16.2%), increased to 23.5% at 12 months. Our data analysis showed that the age, as well as the employment of temporary clipping during surgery, affected the overall outcome. CONCLUSION: Management of poor-grade aSAH patients has been dramatically changed. Microsurgical clipping provides promising results in carefully selected younger patients.

Greek reflections on global neurosurgery.

Introduction: Large populations in middle- and low-income countries in Africa, Asia, and Central and South Americas face a dramatic neurosurgical crisis. However, large social groups in high-income countries face similar limited access to neurosurgical services. Proper identification of such a problem, analysis of the underlying causes, and proposal of potential solutions may not only address the problem at a national level, but may also provide valuable reflections on the efficient management of global neurosurgical crisis. Research question: To evaluate if special social groups face similar problems in Greece. Material and methods: The structure of the Greek health system was examined. The national census along with the registry of practicing neurosurgeons of the Greek National Society, as well as the national health map were searched. Results: A series of socio-economic factors, language barriers, cultural and religion differences, geographical barriers, the COVID-19 pandemic aftermath, along with the inherent malfunctioning of the Greek health system have led to this national neurosurgical crisis. Discussion and conclusion: An extensive redrawing of the Greek health map, reorganization of the national health system, along with adaptation of all recent advances in telemedicine may alleviate the health burden in these populations. The results of this local reformation may be applied to a global level for managing the ongoing health crisis. Moreover, the development of a European taskforce by the European Association of Neurosurgical Societies (EANS) may well facilitate the development of realistic and efficacious global solutions, and contribute to the global effort for providing high-quality neurosurgical services worldwide.

Critical Assessment of the Guidelines-Based Management of Severe Traumatic Brain Injury with the Appraisal of Guidelines for Research and Evaluation II.

BACKGROUND: Severe traumatic brain injury constitutes a clinical entity with complex underlying pathophysiology. Management of patients with severe traumatic brain injury is guided by Clinical Practice Guidelines and Consensus Statements (CPG and CS). The published CPG and CS vary in quality, comprehensiveness, and clinical applicability. The value of critically assessing CPG and CS cannot be overemphasized. The aim of our study was to assess the quality of the published CPG and CS, based on the Appraisal of Guidelines for Research and Evaluation II instrument. METHODS: A systematic search was performed in PubMed, Scopus, Embase, and Web of Science focusing on guidelines and consensi about severe traumatic brain injury . The search terms used were "traumatic brain injury," "TBI," "brain injury," "cerebral trauma," "head trauma," "closed head injury," "head injury," "guidelines," "recommendations," "consensus" in any possible combination. The search period extended from 1964 to 2021 and was limited to literature published in English. The eligible studies were scored by 4 raters, using the Appraisal of Guidelines for Research and Evaluation II instrument. The inter-rater agreement was assessed using the Cronbach's alpha. RESULTS: Twelve CPG and CS were assessed. Overall, the study by Carney et al. was the most Appraisal of Guidelines for Research and Evaluation II compliant study. In general, the domains of clarity of presentation, and scope and purpose, achieved the highest scores. The lowest inter-rater agreement in our analysis was "fair." CONCLUSIONS: The purpose of our study for assessing the quality of CPG and CS was served. We present the strong and weak points of CPG and CS. Our findings support the idea of periodically updating guidelines and improving their rigor of development.

Bilateral Tapia's syndrome secondary to cervical spine injury: a case report and literature review.

A 24-year-old man presented with bilateral Tapia's syndrome (TS) after a traumatic cervical spine injury, manifested by apraxia of the hypoglossal and recurrent laryngeal nerves. The initial presentation was a profound inability to maintain upper respiratory airway patency due to bilateral vocal cord paralysis, accompanied by impairment of swallowing and loss of speech. The diagnosis was based on clinical grounds and verified by endoscopic laryngoscopy. A C7 corpectomy was performed for stabilizing the cervical spine, while conservative treatment with steroids was reserved for the TS. Over the following six months, there was complete resolution of the symptoms.

Valve-controlled chronic subdural hematoma drainage: A feasibility study.

Introduction: Pneumocephalus after chronic subdural hematoma (CSDH) evacuation is a potential predictor of hematoma recurrence. Research question: To study the feasibility and safety of a novel CSDH evacuation technique using a valve-controlled method to avoid pneumocephalus. Material and methods: In a retrospective case series, we evacuated CSDH using very low-pressure valve-controlled drains and recorded the neurological, radiological, and functional outcomes. Patients with primary CSDH, without previous neurosurgical intervention, and who did not receive antiplatelet or anticoagulant therapy the week prior to the index surgery, were included in the study. Exclusion criteria were the evacuation with other treatment techniques and incomplete data files. Patients were assessed according to the Bender grading system to record the neurological status. The hematoma volume was estimated using the formula for ellipsoid volumes. Results: Thirty-six patients with a mean age of 73 years (±9 years) fulfilled our eligibility criteria. Our technique was effective since it decreased the CSDH volume from 141 â€‹ml (IQR 97 â€‹ml) to 20.6 â€‹ml (IQR 26.59 â€‹ml; p â€‹< â€‹0.001) and improved the neurological status according to the Bender grading system from two (IQR 0.25) to 1 (IQR 0). However, pneumocephalus and hematoma recurrence occurred in one case each (2.8%). At six months, all patients returned to their previous status, except for two patients (5.6%) who died due to irrelevant pathologies. Conclusions: Valve-controlled CSDH evacuation aiming to decrease the postoperative pneumocephalus and hematoma recurrence constitutes an effective and safe alternative. However, larger randomized controlled studies are required to establish its role in CSDH management.

Late-Onset Cervical Pseudomeningocele Following Ossification of the Posterior Longitudinal Ligament Surgery Successfully Treated With a Lumboperitoneal Shunt.

Pseudomeningocele (PMC) is a rare complication of anterior cervical procedures resulting in pain, headaches, nerve root entrapment, and in rare cases, spinal cord compression. Here we present a 57-year-old male with increasing myelopathy due to late-onset PMC that developed two years following a 360-degree cervical surgery for ossification of the posterior longitudinal ligament (OPLL). In this case, the PMC was successfully treated with a lumboperitoneal shunt. A 57-year-old male presented with worsening symptoms and signs of cervical myelopathy. He had undergone a multilevel anterior corpectomy/fusion (ACCF), along with posterior fusion, two years earlier for severe ossification of the posterior longitudinal ligament (OPLL). Now presenting with increased myelopathy, his cervical spine MRI demonstrated a PMC in the perivertebral space, extending to and compressing the anterior cervical cord. Following a lumboperitoneal shunt insertion, the patient's myelopathy resolved.  Acute, subacute, or chronic postoperative cervical pseudomeningoceles (PMC) may be readily managed with a lumboperitoneal shunt insertion.

Gender differences in work-life balance of European neurosurgeons.

Introduction: Neurosurgery is one of the most demanding medical specialties. For neurosurgeons, balancing professional activity with personal life can be challenging. Research question: To evaluate gender differences in contribution of neurosurgeons in the household and child-rearing, as well as their impact on personal life and career. Material and methods: An anonymous, electronic, 59-item web-based survey was administered to National Neurosurgical Societies of Europe, and European Member Societies of the European Association of Neurosurgical Societies (June-October 2021). Results: A total of 205 European neurosurgeons (87 females and 118 males, mean age 40.7) are included in our survey. In neurosurgery, females are significantly more likely to be alone (37.9%), while males are significantly more likely to have children (66.9%). In terms of household efforts, females spend more time than males on the same tasks. Most participants (71.2%) view gender issues as a disadvantage in career pursuing. Women feel less accepted (54.3%) and having fewer opportunities (58.6%), while men believe that pregnancy/child-rearing (65.8%) and having many roles (51.3%) are the main obstacles. Both genders (77.6%) favor more convenient working conditions for young parents.Discussion and Conclusion. In our study we found that, women neurosurgeons take more responsibilities at home, especially in the child-rearing years. Female neurosurgeons are more likely to live alone or stay childless more often compared to their male colleagues. Supportive facilities, flexible programs, universal life policies and presumably curbing of the social stereotypes are of importance to overcome gender inequities that women are still facing in neurosurgery.

Critical Appraisal of Randomized Controlled Trials on Unruptured Brain Arteriovenous Malformations.

OBJECTIVE: Brain arteriovenous malformations management remains controversial despite the numerous, available treatment options. Randomized controlled trials (RCTs) theoretically provide the strongest evidence for the assessment of any therapeutic intervention. However, poorly designed RCTs may be associated with biases, inaccuracies, and misleading conclusions. The purpose of our study is to assess reporting transparency and methodological quality of the existing RCTs. METHODS: A search was performed in the PubMed, Scopus, Embase, clinicaltrials.gov, and Cochrane databases. The search was limited to English literature. We included all published RCTs reporting on the management of unruptured brain arteriovenous malformations. The eligible studies were evaluated by 5 blinded raters with the CONsolidated Standards of Reporting Trials 2010 statement and the risk-of-bias 2 tool. The inter-rater agreement was assessed with the Fleiss' Kappa. RESULTS: A randomized trial of unruptured brain arteriovenous malformations (ARUBA) and treatment of brain arteriovenous malformations (TOBAS) trials were evaluated. ARUBA achieved high CONsolidated standards of reporting trials compliance, while TOBAS showed a moderate one. In ARUBA the introduction, discussion, and other information sections reached the highest compliance rate (80%-86%). The lowest rates were recorded in the results and the methods (62% and 73%, respectively). The inter-rater agreement was moderate to substantial (54.1% to 78.4%). All the examined studies demonstrated a high risk of bias, mainly related to ill-defined intended interventions, missing outcome data, and selection of the reported results. CONCLUSIONS: Our study confirmed the high risk of bias mainly attributed to several protocol violations, deviations, minimal external validity and selection, attrition, and allocation biases of the ARUBA trial. Analysis of the TOBAS trial revealed a moderate overall reporting clarity and a high risk of bias.

Methodological assessment of guidelines for the diagnosis and management of cerebral vasospasm using the AGREE-II tool.

OBJECTIVE: Rupture of an intracranial aneurysm is the most common cause of spontaneous subarachnoid hemorrhage. Despite the recent advances in its early detection, diagnosis, and proper treatment, the outcome of patients experiencing aneurysmal subarachnoid hemorrhage (aSAH) remains poor. It is well known that cerebral vasospasm is the most troublesome complication of aSAH, while delayed cerebral ischemia related to cerebral vasospasm constitutes the major cause of unfavorable outcomes in patients with aSAH. The need for evidence-based guidelines is of great importance for the prevention, early detection, and efficient management of aSAH-induced vasospasm. Moreover, guidelines provide young physicians with a valuable tool for practicing defensible medicine. However, the methodology, clinical applicability, reporting clarity, and biases of guidelines must be periodically assessed. In this study, the authors sought to assess the reporting clarity and methodological quality of published guidelines and recommendations. METHODS: A search was performed in the PubMed, Scopus, and Web of Science databases. The search terms used were "clinical practice guidelines," "recommendations," "stroke," "subarachnoid hemorrhage," and "vasospasm" in all possible combinations. The search period extended from 1964 to September 2021 and was limited to literature published in the English language. All published guidelines and recommendations reporting on the diagnosis and management of vasospasm were included. Studies other than those reporting guidelines and recommendations were excluded. The eligible studies were evaluated by three blinded raters, employing the Appraisal of Guidelines for Research & Evaluation II (AGREE-II) analysis tool. RESULTS: A total of 10 sets of guidelines were evaluated in this study. The American Heart Association/American Stroke Association issued guidelines found to have the highest methodological quality and reporting clarity, followed by the European Stroke Organization guidelines and the English edition of the Japanese guidelines issued by the Japanese Society on Surgery for Cerebral Stroke. The interrater agreement was moderate in the current analysis. CONCLUSIONS: These findings support the idea that improvement of currently existing guidelines is feasible in the following domains: the rigor of guidelines and recommendations development, clinical applicability, editorial independence, and stakeholder involvement. Furthermore, periodic updating of published guidelines requires improvement in the future.

Intermediate surgical outcome in patients suffering poor-grade aneurysmal subarachnoid hemorrhage. A single center experience.

BACKGROUND: It is known that patients suffering poor-grade aneurysmal subarachnoid hemorrhage (aSAH) have a dismal prognosis. The importance of early intervention is well established in the pertinent literature. Our aim was to assess the functional outcome and overall survival of these patients undergoing surgical clipping. MATERIAL AND METHODS: In the current retrospective study we included all consecutive poor-grade patients after spontaneous SAH who presented at our institution over an eight-year period. All participants suffering SAH underwent brain CT angiography (CTA) to identify the source of hemorrhage. We assessed the severity of hemorrhage according to the Fisher grade classification scale. All patients were surgically treated. The functional outcome was evaluated six months after the onset with the Glasgow Outcome Scale. Finally, we performed logistic and Cox regression analyses to identify potential prognostic risk factors. RESULTS: Our study included twenty-three patients with a mean age of 53 years. Five (22%) patients presented with Hunt and Hess grade IV, and eighteen (78%) with grade V. The mean follow-up was 15.8 months, while the overall mortality rate was 48%. The six-month functional outcome was favorable in 6 (26%) patients. The vast majority of our patients died between the 15th and the 60th post-ictal days. We did not identify any statistically significant prognostic factors related to the patient's outcome and/or survival. CONCLUSIONS: Poor-grade aSAH patients may have a favorable outcome with proper surgical management. Large-scale studies are necessary for accurately outlining the prognosis of this entity, and identifying parameters that could be predictive of outcome.

Guidelines on the use of external ventricular drain and its associated complications: do we "AGREE II"?

Insertion of an external ventricular drain is a common procedure used in everyday practice by neurosurgeons all around the world. It consists of the placement of an external ventricular drain (EVD) into the ventricular system providing the ability to measure intracranial pressure, and also divert the flow of cerebrospinal fluid (CSF) in a variety of pathological conditions. The most common complication is infection, and it may result in devastating consequences and negatively affect the outcome of these patients. The Infectious Diseases Society of America (IDSA), the Neurocritical Care Society (NCS), and The Society for Neuroscience in Anesthesiology & Critical Care (SNACC) have published recommendations for the management of EVD-Associated Ventriculitis. The objective of this study was to assess the methodological quality and reporting clarity of these recommendations using the AGREE-II tool. We found that the overall quality of the published clinical practice guidelines is acceptable. However, continuous updates and external validation should be implemented.

The Impact of the Coronavirus Pandemic on European Neurosurgery Trainees.

BACKGROUND: The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has placed tremendous strain on the national health care systems throughout Europe. As a result, there has been a significant influence on residents' education. We surveyed European neurosurgery residents to estimate the magnitude of the pandemic's impact on neurosurgical training. METHODS: An anonymous, voluntary, 44-question, web-based survey was administered to European neurosurgical residents from November 2, 2020, to January 15, 2021, by e-mail invitation. Close-ended, multiple-choice questions were used to examine the perspectives of neurosurgical trainees of different training programs in Europe regarding the pandemic's impact on education, as well as to evaluate the online webinars as a sufficient alternative educational tool, and their future role. RESULTS: The total number of participants was 134 from 22 European countries. Nearly 88.8 % of respondents reported that the pandemic had a negative influence on their education. A statically significant decrease in surgical exposure, outpatient clinic involvement, and working hours was observed (P < 0.05). Webinars, although widely disseminated, were not considered as a sufficient training alternative. CONCLUSIONS: The SARS-CoV-2 pandemic had a significant impact on neurosurgical training. During the last year, with the outbreak of the pandemic, formal training education was heavily compromised. Online webinars do not seem to be a sufficient alternative, and some trainees estimate that a whole year of training has been compromised. Our current data have to be cautiously considered for possibly reorganizing the whole training experience. The pandemic may well function as a stimulus for optimizing neurosurgical training.

Bursitis of the coccyx in an adult with rheumatoid arthritis mimicking a sacrococcygeal meningocele.

BACKGROUND: Bursitis is a chronic inflammatory condition characterized by the deposition of cholesterol, macrophage infiltration, and bursal wall calcification. Bursitis is, however, rarely found in the sacrococcygeal region where it may present as a space-occupying mass. CASE DESCRIPTION: A 64-year-old male with rheumatoid arthritis presented with 3 years' duration of difficulty sitting and walking due to a soft-tissue mass involving the coccyx region. Once the patient's MR demonstrated a cystic lesion with erosion of the coccyx, the patient underwent gross total resection of the lesion that proved to be pathologically consistent with bursitis. Postoperatively, the patient's complaints fully resolved. CONCLUSION: Bursitis may present as a soft-tissue tumor-like lesion in the coccyx that favorably responds to gross total surgical excision.

The Role of the T2-FLAIR Mismatch Sign as an Imaging Marker of IDH Status in a Mixed Population of Low- and High-Grade Gliomas.

Our study evaluated the role of the T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign in detecting isocitrate dehydrogenase (IDH) mutations based on a mixed sample of 24 patients with low- and high- grade gliomas. The association between the two was realized using univariate and multivariate logistic regression analysis. There was a substantial agreement between the two raters for the detection of the T2-FLAIR mismatch sign (Cohen's kappa coefficient was 0.647). The T2-FLAIR mismatch sign when co-registered with the degree of tumor homogeneity were significant predictors of the IDH status (OR 29.642; 95% CI 1.73-509.15, p = 0.019). The probability of being IDH mutant in the presence of T2-FLAIR mismatch sign was as high as 92.9% (95% CI 63-99%). The sensitivity and specificity of T2-FLAIR mismatch sign in the detection of the IDH mutation was 88.9% and 86.7%, respectively. The T2-FLAIR mismatch sign may be an easy to use and helpful tool in recognizing IDH mutant patients, particularly if formal IDH testing is not available. We suggest that the adoption of a protocol based on imaging and histological data for optimal glioma characterization could be very helpful.

Arteriovenous Malformations: Congenital or Acquired Lesions?

OBJECTIVE: Arteriovenous malformations (AVMs) were believed to be congenital. However, an increasing number of de novo AVM cases have questioned this doctrine. METHODS: A consensus meeting of international experts attempted to establish a consensus on the nature of these relatively rare but challenging vascular lesions. In addition, an extensive search of the subject was performed using the PubMed medical database. RESULTS: All participants agreed that genetic factors may play a role in the pathogenesis of AVMs. All but 1 participant believed that an underlying genetic predisposition may be detected later on in a patient's life, whereas genetic variations may contribute to sporadic AVM formation. The presence of genetic variations alone may not be enough for an AVM formation. A second hit is probably required. This consensus opinion is also supported by our literature search. CONCLUSIONS: We discuss the literature on the genetics of AVMs and compare it with the consensus meeting outcomes. The congenital or noncongenital character of intracranial AVMs has an impact on the understanding their biological behavior, as well as their efficient short-term and long-term management.

Complications Associated with Surgery for Thoracic Disc Herniation: A Systematic Review and Network Meta-Analysis.

BACKGROUND: A systematic review and network meta-analysis (Prospero ID CRD42018106936) were performed. OBJECTIVE: The selection of the appropriate surgical approach for the management of thoracic disc herniation (TDH) is often challenging because of the frequency and variability of the associated complications. We evaluated the safety of the surgical approaches for TDH by estimating the mortality (Q1) and morbidity (Q2), and frequency of the most common complications (Q3). METHODS: We searched the medical literature for randomized controlled trials and observational studies reporting on the management of TDH. Postoperative complications were the outcome of interest. The absolute and relative risk estimates, along with the rank probability scores, were estimated for each approach, through a network meta-analysis. The results were read in the light of the quality of the available evidence. RESULTS: Fifteen studies with a total of 1036 patients fulfilled our eligibility criteria. Three deaths were reported. The overall morbidity was as high as 29%, largely attributed to medical (21%; 95% confidence interval [CI], 10%-38%), surgical site (11%; 95% CI, 5%-22%), cerebrospinal fluid-related (8%; 95% CI, 3%-8%), and neurologic complications (5%; 95% CI, 1%-24%). The anterior and lateral approaches were associated with a higher risk for medical and surgical complications compared with the posterolateral approach. CONCLUSIONS: Surgery for TDH is associated with minimal mortality but significant morbidity, with large variations among the available approaches. An understanding of the perioperative complications rates is important to develop complication avoidance strategies and to aid accurate patient-to-doctor communication.

Recurrence Is Associated With Body Mass Index in Patients Undergoing a Single-Level Lumbar Disc Herniation Surgery.

BACKGROUND: The aim of the study was to assess the body mass index (BMI) and other risk factors associated with lumbar disc herniation (LDH) and clinical outcomes, in patients who undergo surgery for single-level LDH. METHODS: This was a retrospective cohort study, affecting patients that underwent surgery for single-level LDH attending our hospital between July 2009 and January 2016. The mean follow-up period was 3.5 years (1 - 8 years). To maintain adequately sized groups for analysis, level L2-L3 and L3-L4 herniations were grouped as upper disc levels (group A) and level L4-L5 (group B) and L5-S1 (group C) herniations were analyzed individually. Disk herniation was graded on T2-weighted sagittal magnetic resonance images by using a five-point scale. Pain assessment was made using the visual analog scale (VAS). RESULTS: Two hundred fifty-six (256) patients met study inclusion criteria. There were 138 males (53.9%) with a mean age of 55.3 ± 12.9 years (range, 30 - 77). The association between A, B and C groups was analyzed, based on criteria such as age, sex, BMI, surgical techniques, diabetes, size of herniated disc, preoperative VAS, length of hospital stay, drop foot on admission, smoking, family history and history of injury to the lumbar spine, location of herniated disc (far lateral) and use of steroids. We found a statistically significant factor between groups in BMI (P = 0.006), family history (P = 0.001), location (far lateral) (P = 0.003) and history of injury to the lumbar spine (P = 0.003). CONCLUSIONS: There may be an association between severity of disc degeneration and BMI (overweight and obese adults). Furthermore, spine and neurosurgeons should be aware that BMI might be related to patients' outcome.

Multimodality treatment of intradural extramedullary Ewing's sarcomas. A systematic review.

Ewing's sarcoma (ES) is an aggressive bone and soft tissue sarcoma that usually affects adolescents and young adults. ES occasionally presents as an intradural-extramedullary lesion of the spine. Our aim was to study the role of the multimodality treatment on the survival (overall survival, recurrence-free survival, and metastasis-free survival) of patients with intradural-extramedullary Ewing's sarcoma. Pubmed, EMBASE, Scopus, Web of Science, Cochrane Reviews were searched up to January 2017, using as mesh terms "intradural extramedullary", "Ewing's sarcoma", AND "treatment". The multidisciplinary treatment was recorded in binary variables under the headings of "surgery", "chemotherapy" and "radiotherapy". We also recorded three time-to-event variables, including death, recurrence, and metastasis. We performed survival analysis for all potential combinations. Twenty articles with twenty-three patients were eligible for the current review. The survival curves of GTR did not differ from the equivalent of STR regarding survival (p=0.098), recurrence-free survival (p=0.318), and metastasis-free survival (p=0.089). Patients who received chemotherapy enjoyed longer survival regarding overall survival (p<0.05), recurrence-free survival (p<0.05), and metastasis-free survival (p<0.05), when compared to those who did not receive chemotherapy. Their overall survival of patients who had radiotherapy was marginally superior to those who did not receive (p=0.0653). However, their recurrence-free survival (p<0.05), and metastasis-free survival (p<0.05) were significantly improved in comparison to the latter. In conclusion, the multimodality treatment is mandatory for the management of patients with intradural extramedullary Ewing's sarcomas, with surgery assisting in the diagnosis and decompression the neural elements. However, it is chemotherapy that improves survival, recurrence-free survival, and metastasis-free survival. Radiotherapy is reserved as an adjuvant therapy in the local control, especially in cases with subtotal tumour resection.