Genome-wide association analysis identifies a susceptibility locus for sporadic vestibular schwannoma at 9p21.

Vestibular schwannomas are benign nerve sheath tumours that arise on the vestibulocochlear nerves. Vestibular schwannomas are known to occur in the context of tumour predisposition syndromes NF2-related and LZTR1-related schwannomatosis. However, the majority of vestibular schwannomas present sporadically without identification of germline pathogenic variants. To identify novel genetic associations with risk of vestibular schwannoma development, we conducted a genome-wide association study in a cohort of 911 sporadic vestibular schwannoma cases collated from the neurofibromatosis type 2 genetic testing service in the north-west of England, UK and 5500 control samples from the UK Biobank resource. One risk locus reached genome-wide significance in our association analysis (9p21.3, rs1556516, P = 1.47 × 10-13, odds ratio = 0.67, allele frequency = 0.52). 9p21.3 is a genome-wide association study association hotspot, and a number of genes are localized to this region, notably CDKN2B-AS1 and CDKN2A/B, also referred to as the INK4 locus. Dysregulation of gene products within the INK4 locus have been associated with multiple pathologies and the genes in this region have been observed to directly impact the expression of one another. Recurrent associations of the INK4 locus with components of well-described oncogenic pathways provides compelling evidence that the 9p21.3 region is truly associated with risk of vestibular schwannoma tumorigenesis.

Predictors of future haemorrhage from cerebral cavernous malformations: a retrospective cohort study.

Cerebral cavernous malformations (CCMs) are commonly diagnosed, with a low reported rate of haemorrhage on long-term follow-up. The identification of factors predictive of future haemorrhage risk would assist in guiding the management of patients with CCM. The aim of this study was to identify variables associated with haemorrhage, and calculate haemorrhage risk in CCM. We conducted a retrospective study of patients diagnosed with a CCM, managed at a specialist tertiary neuroscience centre (2007-2019). The primary outcome was symptomatic haemorrhage, and secondary outcomes were variables associated with increased risk of haemorrhage, using multivariable Cox regression analysis. Included were 545 patients, with 734 confirmed cavernomas. Median age at diagnosis was 47 (interquartile range [IQR] 35-60), with a median follow-up duration after diagnosis of 46 months (IQR 19-85). Of the patients, 15.0% had multiple lesions (N = 82/545). Symptomatic presentation was observed in 52.5% of patients (N = 286/545). The annual haemorrhage rate was 1.00% per lesion-year (25 events in 2512 lesion-years), and higher in those with symptoms at presentation (1.50% per lesion-year, 22 events vs 0.29%, 3 events, P < 0.001). The variables associated with symptomatic haemorrhage were increased size (hazard ratio [HR] 1.04, 95% confidence interval [CI] 1.01-1.07, P = 0.004), eloquent location (HR 2.63, 95% CI 1.12-6.16, P = 0.026), and symptomatic haemorrhage at presentation (HR 5.37, 95% CI 2.40-11.99, P < 0.001). This study demonstrated that CCMs have a low haemorrhage rate. Increased size, eloquent location, and haemorrhage at presentation appear to be predictive of a higher risk of haemorrhage, and could be used to stratify management protocols.

Multi-layered repair of high-flow CSF fistulae following endoscopic skull base surgery without nasal packing or lumbar drains: technical refinements to optimise outcome.

AIMS: Post-operative CSF leak remains a significant problem following endoscopic skull base surgery, particularly when there is a high-flow intra-operative CSF leak. Most skull base repair techniques are accompanied by the insertion of a lumbar drain and/or the use of nasal packing which have significant shortcomings. Our aim was to review the results of a large series of endoscopic skull base cases where a high-flow intra-operative CSF leak rate was encountered and repaired to assess if modifications in technique could reduce the post-operative CSF leak rate. METHODS: A retrospective review of a prospectively maintained database of skull base cases performed by a single surgeon over a 10-year period was performed. Data regarding patient demographics, underlying pathology, skull base repair techniques and post-operative complications were analysed. RESULTS: One hundred forty-two cases with high-flow intra-operative CSF leak were included in the study. The most common pathologies were craniopharyngiomas (55/142, 39%), pituitary adenomas (34/142, 24%) and meningiomas (24/142, 17%). The CSF leak rate was 7/36 (19%) when a non-standardised skull base repair technique was used. However, with the adoption of a standardised, multi-layer repair technique, the post-operative CSF leak rate decreased significantly (4/106, 4% vs. 7/36, 19%, p = 0.006). This improvement in the rate of post-operative CSF leak was achieved without nasal packing or lumbar drains. CONCLUSION: With iterative modifications to a multi-layered closure technique for high-flow intra-operative CSF leaks, it is possible to obtain a very low rate of post-operative CSF leak, without lumbar drains or nasal packing.

Radiological follow-up of endovascularly treated intracranial aneurysms: a survey of current practice in the UK and Ireland.

PURPOSE: Due to the risk of intracranial aneurysm (IA) recurrence and the potential requirement for re-treatment following endovascular treatment (EVT), radiological follow-up of these aneurysms is necessary. There is little evidence to guide the duration and frequency of this follow-up. The aim of this study was to establish the current practice in neurosurgical units in the UK and Ireland. METHODS: A survey was designed with input from interventional neuroradiologists and neurosurgeons. Neurovascular consultants in each of the 30 neurosurgical units providing a neurovascular service in the UK and Ireland were contacted and asked to respond to questions regarding the follow-up practice for IA treated with EVT in their department. RESULTS: Responses were obtained from 28/30 (94%) of departments. There was evidence of wide variations in the duration and frequency of follow-up, with a minimum follow-up duration for ruptured IA that varied from 18 months in 5/28 (18%) units to 5 years in 11/28 (39%) of units. Young patient age, previous subarachnoid haemorrhage and incomplete IA occlusion were cited as factors that would prompt more intensive surveillance, although larger and broad-necked IA were not followed-up more closely in the majority of departments. CONCLUSIONS: There is a wide variation in the radiological follow-up of IA treated with EVT in the UK and Ireland. Further standardisation of this aspect of patient care is likely to be beneficial, but further evidence on the behaviour of IA following EVT is required in order to inform this process.

Sinonasal outcomes associated with the use of BioGlue® in endoscopic transsphenoidal pituitary surgery.

Concerns have been raised in the neurosurgical literature regarding the use of BioGlue®, a tissue sealant initially developed for cardiothoracic surgery, due to reports of an increased incidence of infection, wound breakdown, and CSF fistulae. The aim of this study was to determine if the use of BioGlue® in skull base repair impacts on the sinonasal outcomes and the incidence of post-operative infection following endoscopic pituitary surgery. SNOT-22 questionnaires were completed pre-operatively, and at 6, 12, and 24 weeks post-operatively by 50 patients. Pre- and post-operative MRI scans were reviewed and assessed for evidence of post-operative sinusitis. Patient records were consulted to ascertain the incidence of post-operative infection. Repeated measures ANOVA and Wilcoxon signed rank test were used for data analysis. Statistical analysis revealed a significant difference in the mean SNOT-22 scores across the four time intervals (F(1.605, 78.642) = 9.180, p = 0.001). This difference was powered by a deterioration at 6 weeks that recovered completely by 12 weeks. The mean SNOT-22 score in the study cohort at 24 weeks was 16.84/110 (range 0-57, ± 2.04) which was lower than the mean pre-operative score and is consistent with mild rhinosinusitis. There were no cases of post-operative meningitis and 1/50 (2%) case of post-operative sinusitis in this cohort. The use of BioGlue® in endoscopic endonasal pituitary surgery does not appear to lead to significant sinonasal morbidity. Previous concerns about the use of BioGlue® in transcranial procedures may not apply to extradural application of this tissue sealant in endoscopic transnasal surgery.

How I do it: coil extraction and clip reconstruction of a previously coiled giant middle cerebral artery aneurysm.

BACKGROUND: Microsurgical clipping of previously coiled intracranial aneurysms can be technically challenging. We present the case of a 48-year-old female with a partially coiled, previously ruptured giant middle cerebral artery aneurysm and the associated technical nuances. METHODS: We performed a successful coil extraction and clip reconstruction of this aneurysm using an ultrasonic aspirator and stacked fenestrated aneurysm clips. CONCLUSION: Coil extraction and clip occlusion of previously coiled intracranial aneurysms is a complex and technically challenging procedure but can be performed safely and effectively when employed judiciously.

3 Tesla intra-operative MRI as an adjunct to endoscopic pituitary surgery: an early assessment of clinical utility.

BACKGROUND: Extent of resection is a major determinant of outcomes following pituitary surgery. Intra-operative magnetic resonance imaging (iMRI), provides an immediate assessment of the extent of resection, allowing further tumour resection during the same procedure. However, such systems are expensive and significantly increase operative time, prompting some authors to question the additional benefit conferred by iMRI when combined with endoscopy. Our aim was to assess the impact of combining 3 T iMRI with endoscopy in patients with pituitary tumours. METHODS: We retrospectively reviewed a prospectively maintained database to identify patients who underwent iMRI guided endoscopic resection of pituitary tumours between May 2017 and November 2018 (iMRI cohort). This cohort was compared with a pre-iMRI cohort of patients who underwent endoscopic resection of pituitary adenomas. Operative time, extent of resection, control of endocrine disease and post-operative complications were recorded and analysed. RESULTS: Thirty-seven patients were included in each cohort. iMRI facilitated additional tumour resection in 6/37 (16%) of cases. In 4/37 cases (11%), iMRI prompted a return to theatre but no further tumour could be identified. The overall GTR rate, following iMRI was 24/37 (65%) as compared to 21/37 (57%) in the pre-iMRI cohort. Cure of endocrine disease associated with hormonally active tumours was achieved in 9/11 (82%) of cases in the iMRI cohort. The mean operative time in the iMRI cohort was 327 minutes (five hours 27 minutes). CONCLUSIONS: 3 T iMRI provides immediate identification of residual tumour following endoscopic pituitary surgery. This allows for resection of surgically accessible residual disease during the same procedure and is likely to reduce the requirement for later re-intervention. However, the use of iMRI in this setting is associated with significant resource allocation issues which must be considered prior to the widespread adoption of this technique.

Predicting post-operative cerebrospinal fluid (CSF) leak following endoscopic transnasal pituitary and anterior skull base surgery: a multivariate analysis.

BACKGROUND: Post-operative CSF leak is the major source of morbidity following endoscopic transsphenoidal surgery. The purpose of this study was to identify factors associated with post-operative CSF leak in patients undergoing this surgery and facilitate the prospective identification of patients at higher risk of this complication. METHODS: A review of a prospectively maintained database containing details of 270 endoscopic transsphenoidal operations performed by the senior author over a 9-year period was performed. Univariate analysis was performed using the Chi-squared and Fisher's exact tests, as appropriate. A logistic regression model was constructed for multivariate analysis. RESULTS: The rate of post-operative CSF leak in this series was 9%. On univariate analysis, previous surgery, resection of craniopharyngiomas, adenomas causing Cushing's disease and intra-operative CSF leaks were associated with an increased risk of post-operative CSF leak. The use of a vascularised nasoseptal flap and increasing surgical experience were associated with a decreased rate of CSF leak. On multivariate analysis, a resection of tumour for Cushing's disease (OR 5.79, 95% CI 1.53-21.95, p = 0.01) and an intra-operative CSF leak (OR 4.56, 95% CI 1.56-13.32, p = 0.006) were associated with an increased risk of post-operative CSF leak. Increasing surgical experience (OR 0.14, 95% CI 0.04-0.46, p = 0.001) was strongly associated with a decreased risk of post-operative CSF leak. CONCLUSIONS: Increasing surgical experience is a strong predictor of a decreased risk of developing post-operative CSF leak following endoscopic transsphenoidal surgery. Patients with Cushing's disease and those who develop an intra-operative CSF leak should be managed with meticulous skull base repair and close observation for signs of CSF leak post-operatively.

Suprasellar Anterior-Posterior Diameter Optimizes the Use of Intraoperative MRI in Patients Undergoing Endoscopic Pituitary Surgery.

BACKGROUND AND OBJECTIVES: Intraoperative MRI (iMRI) has been demonstrated to improve the extent of resection of pituitary neuroendocrine tumors resected using endoscopic endonasal approaches. We sought to establish if preoperative clinicoradiological parameters could be used to predict which patients are most likely to benefit from iMRI and thus allow more efficient use of this technology. METHODS: A prospectively maintained surgical database of all endoscopic pituitary tumor resections with iMRI guidance performed between May 2017 and September 2023 was accessed. Data were collected on clinical and radiological parameters that may predict reintervention after iMRI. Logistic regression models were constructed to assess the relationship between predictor variables and reintervention after iMRI. RESULTS: Seventy-three patients were included in the study. After review of the iMRI, 24/73 (33%) patients underwent surgical reintervention. The combined rate of gross total resection/near total resection was 64/73 (88%). The rate of biochemical cure of endocrine disease after surgery for a hormonally active tumor was 15/21 (71%). On univariate logistic regression analysis, the only factor significantly associated with reintervention after iMRI was the suprasellar anterior-posterior diameter (odds ratio 1.1, 95% CI 1.01-1.2, P = .030). CONCLUSION: Suprasellar anterior-posterior diameter ≥15 mm predicts the requirement for reintervention after endoscopic resection of pituitary neuroendocrine tumor. Use of this easily obtained radiological parameter will allow iMRI to be used in those patients who are most likely to benefit.

Reported Hearing Outcome Measures Following Stereotactic Radiosurgery for Vestibular Schwannoma: A Scoping Review.

Background Evidence on hearing outcome measures when assessing hearing preservation following stereotactic radiosurgery (SRS) for adults with vestibular schwannoma (VS) has not previously been collated in a structured review. Objective The objective of the present study was to perform a scoping review of the evidence regarding the choice of hearing outcomes and other methodological characteristics following SRS for adults with VS. Methods The protocol was registered in the International Platform of Registered Systematic Review and Meta-Analysis Protocols (INPLASY) and reported according to the Preferred Reporting Items for Systematic Review and Meta-Analyses extension guidelines for scoping reviews. A systematic search of five online databases revealed 1,591 studies, 247 of which met the inclusion criteria. Results The majority of studies ( n = 213, 86%) were retrospective cohort or case series with the remainder ( n = 34, 14%) prospective cohort. Pure-tone audiometry and speech intelligibility were included in 222 (90%) and 158 (64%) studies, respectively, often summarized within a classification scheme and lacking procedural details. Fifty-nine (24%) studies included self-report measures. The median duration of follow-up, when reported, was 43 months (interquartile range: 29, 4-150). Conclusion Evidence on hearing disability after SRS for VS is based on low-quality studies which are inherently susceptible to bias. This review has highlighted an urgent need for a randomized controlled trial assessing hearing outcomes in patients with VS managed with radiosurgery or radiological observation. Similarly, consensus and coproduction of a core outcome set to determine relevant hearing and communication outcome domains is required. This will ensure that patient priorities, including communication abilities in the presence of background noise and reduced participation restrictions, are addressed.

Radiation-Associated Vestibular Schwannomas: Case Series and Literature Review.

OBJECTIVES: Radiation treatment, particularly at a young age, creates theoretical risk for long-term adverse radiation effects, including the development of malignancy. The literature is sparse on radiation-induced vestibular schwannomas (VSs). METHODS: A retrospective review was performed for cases of suspected radiation-induced VS at 2 high-volume centers. Only cases where radiation included coverage of the posterior fossa were included with those diagnosed within 3 years of radiation treatment being excluded. Patient and tumor characteristics were collected. A systematic literature review was also performed for any previously published series on radiation-induced VS. RESULTS: Eight cases of radiation-induced VS were identified with a median follow-up 125 months (range 7-131). The median age at incident radiation was 15 years (range 2-46). The median age at VS diagnosis was 57 years (range 26-83) with median interval from radiation to diagnosis of 51-years (range 15-66). The median tumor size was 6 mm (range 3-21). Two patients underwent surgical resection. Lesions were described as soft and highly vascular, with medium to high adherence to the facial nerve. Five articles with a total of 52 patients were identified, median age at VS diagnosis was 42-years (range 23-73) with a median interval from radiation to diagnosis of 19 years (range 15-23). CONCLUSIONS: The development of VS following radiation exposure appears rare and our understanding of the condition remains incomplete. Further studies are required to determine the best management of these patients and determine whether there is a causative relationship between radiation exposure and the development of VS.

Does tumoral cavernous carotid stenosis predict an increased risk of future stroke in skull base meningiomas?

OBJECTIVE: Skull base meningiomas (SBMs) involving the cavernous sinus encase the internal carotid artery (ICA) and may lead to stenosis of the vessel. Although ischemic stroke has been reported in the literature, there are to the authors' knowledge no reported studies quantifying the risk of stroke in these patients. The authors aimed to determine the frequency of arterial stenosis in patients with SBMs that encase the cavernous ICA and to estimate the risk of ischemic stroke in these patients. METHODS: Records of all patients with SBM encasing the ICA whose cases were managed by the skull base multidisciplinary team at Salford Royal Hospital between 2011 and 2017 were reviewed using a two-stage approach: 1) clinical and radiological strokes were identified from electronic patient records, and 2) cases were reviewed to examine the correlation between ICA stenosis associated with SBM encasement and anatomically related stroke. Strokes that were caused by another pathology or did not occur in the perfusion territory were excluded. RESULTS: In the review of patient records the authors identified 118 patients with SBMs encasing the ICA. Of these, 62 SBMs caused stenosis. The median age at diagnosis was 70 (IQR 24) years, and 70% of the patients were female. The median follow-up was 97 (IQR 101) months. A total of 13 strokes were identified in these patients; however, only 1 case of stroke was associated with SBM encasement, which occurred in the perfusion territory of a patient without stenosis. Risk of acute stroke during the follow-up period for the entire cohort was 0.85%. CONCLUSIONS: Acute stroke in patients with ICA encasement by SBMs is rare despite the propensity of these tumors to stenose the ICA. Patients with ICA stenosis secondary to their SBM did not have a higher incidence of stroke than those with ICA encasement without stenosis. The results of this study demonstrate that prophylactic intervention to prevent stroke is not necessary in ICA stenosis secondary to SBM.

The clinical, genetic, and immune landscape of meningioma in patients with NF2-schwannomatosis.

NF2-schwannomatosis is the most common genetic predisposition syndrome associated with meningioma. Meningioma in NF2-schwannomatosis is a major source of morbidity and mortality. This is due to accumulative tumor burden in patients with synchronous schwannomas and ependymomas, sometimes including complex collision tumors. Balancing the impact of multiple interventions against the natural history of various index tumors, and the ongoing risk of de novo tumors over an individual's lifetime makes decision-making complex. The management of any given individual meningioma is often different from a comparable sporadic tumor. There is typically a greater emphasis on conservative management and tolerating growth until a risk boundary is reached, whereby symptomatic deterioration or higher risk from anticipated future treatment is threatened. Management by high-volume multidisciplinary teams improves quality of life and life expectancy. Surgery remains the mainstay treatment for symptomatic and rapidly enlarging meningioma. Radiotherapy has an important role but carries a higher risk compared to its use in sporadic disease. Whilst bevacizumab is effective in NF2-associated schwannoma and cystic ependymoma, it has no value in the management of meningioma. In this review, we describe the natural history of the disease, underlying genetic, molecular, and immune microenvironment changes, current management paradigms, and potential therapeutic targets.

Increased Circulating Chemokines and Macrophage Recruitment in Growing Vestibular Schwannomas.

BACKGROUND: There is evidence that macrophage infiltration in the tumor microenvironment promotes vestibular schwannoma (VS) growth. Efficacy of bevacizumab in NF2-associated VS demonstrates the value of therapies targeting the microvascular tumor microenvironment, and tumor-associated macrophages (TAMs) may represent another druggable target. OBJECTIVE: To characterize the relationship between growth, TAM infiltration, and circulating monocyte chemokines in a large cohort of patients with VS. METHODS: Immunostaining for Iba1 (macrophages), CD31 (endothelium), and fibrinogen (permeability) was performed on 101 growing and 19 static sporadic VS. The concentrations of monocyte-specific chemokines were measured in the plasma of 50 patients with growing VS and 25 patients with static VS. RESULTS: The Iba1 + cell count was significantly higher in growing as compared with static VS (592 vs 226/×20 HPF, P =<0.001). Similarly, the CD31 + % surface area was higher in growing VS (2.19% vs 1.32%, P = .01). There was a positive correlation between TAM infiltration and VS growth rate, which persisted after controlling for the effect of tumor volume (aR2 = 0.263, P =<0.001). The plasma concentrations of several monocytic chemokines were higher in patients with growing rather than static VS. CONCLUSION: There is a strong positive correlation between TAM infiltration and volumetric growth of VS, and this relationship is independent of tumor size. There is a colinear relationship between TAM infiltration and tumor vascularity, implying that inflammation and angiogenesis are interlinked in VS. Chemokines known to induce monocyte chemotaxis are found in higher concentrations in patients with growing VS, suggestive of a potential pathophysiological mechanism.

Intraoperative diagnosis of facial schwannomas: a multicenter summation of clinical experience, preoperative avoidance, and intraoperative management protocol.

OBJECTIVE: Preoperative differentiation of facial nerve schwannoma (FNS) from vestibular schwannoma (VS) can be challenging, and failure to differentiate between these two pathologies can result in potentially avoidable facial nerve injury. This study presents the combined experience of two high-volume centers in the management of intraoperatively diagnosed FNSs. The authors highlight clinical and imaging features that can distinguish FNS from VS and provide an algorithm to help manage intraoperatively diagnosed FNS. METHODS: Operative records of 1484 presumed sporadic VS resections between January 2012 and December 2021 were reviewed, and patients with intraoperatively diagnosed FNSs were identified. Clinical data and preoperative imaging were retrospectively reviewed for features suggestive of FNS, and factors associated with good postoperative facial nerve function (House-Brackmann [HB] grade ≤ 2) were identified. A preoperative imaging protocol for suspected VS and recommendations for surgical decision-making following an intraoperative FNS diagnosis were created. RESULTS: Nineteen patients (1.3%) with FNSs were identified. All patients had normal facial motor function preoperatively. In 12 patients (63%), preoperative imaging demonstrated no features suggestive of FNS, with the remainder showing subtle enhancement of the geniculate/labyrinthine facial segment, widening/erosion of the fallopian canal, or multiple tumor nodules in retrospect. Eleven (57.9%) of the 19 patients underwent a retrosigmoid craniotomy, and in the remaining patients, a translabyrinthine (n = 6) or transotic (n = 2) approach was used. Following FNS diagnosis, 6 (32%) of the tumors underwent gross-total resection (GTR) and cable nerve grafting, 6 (32%) underwent subtotal resection (STR) and bony decompression of the meatal facial nerve segment, and 7 (36%) underwent bony decompression only. All patients undergoing subtotal debulking or bony decompression exhibited normal postoperative facial function (HB grade I). At the last clinical follow-up, patients who underwent GTR with a facial nerve graft had HB grade III (3 of 6 patients) or IV facial function. Tumor recurrence/regrowth occurred in 3 patients (16%), all of whom had been treated with either bony decompression or STR. CONCLUSIONS: Intraoperative diagnosis of an FNS during a presumed VS resection is rare, but its incidence can be reduced further by maintaining a high index of suspicion and undertaking further imaging in patients with atypical clinical or imaging features. If an intraoperative diagnosis does occur, conservative surgical management with bony decompression of the facial nerve only is recommended, unless there is significant mass effect on surrounding structures.

Traumatic compression fractures in thoracic-lumbar junction: vertebroplasty vs conservative management in a prospective controlled trial.

BACKGROUND: Both surgery and conservative management are well established treatments for compression fractures of the thoraco-lumbar spine without neurological compromise. This article aims to compare the outcomes of conservative management to those of vertebroplasty, a relatively safe and simple procedure. METHODS: 102 patients were admitted to our neurosurgical unit between January 2012 and February 2016, presenting with a single-level, post-traumatic A1 or A2 Mager l type fracture, affecting the thoracic-lumbar spine without any neurological deficits. After description of both treatment options, the patients were asked to choose between vertebroplasty or conservative treatment. Accordingly, the patients were allocated into two groups and a prospective non-randomized controlled trial was carried out. The first group (Group A) included 52 patients, treated with bed rest and an orthosis. The second group (Group B) of 50 patients underwent a percutaneous vertebroplasty. Pain intensity (assessed via visual analog scale (VAS)), disability degree (assessed via Oswestry Disability Index), ability to resume work (assessed via Denis work Scale), vertebral body height loss rate, regional kyphosis angle (Cobb's angle), duration of hospitalization and treatment-associated complications, were prospectively recorded in a database and analyzed. Follow ups were planned at 1, 6, and 12 months after the injury. RESULTS: Group B, compared with group A, showed a faster improvement in VAS score as well as functional ability and return to work. Cobb's angle progression was significantly less in the surgical group. Morbidity, mortality, and complication rate were similar and comparable in both groups without a statistical difference (P<0.05) CONCLUSIONS: Vertebroplasty is a safe and effective treatment in post-traumatic thoracic-lumbar fractures compared with conservative management.

Surgical and Endovascular Treatment of Saccular Posterior Inferior Cerebellar Artery Aneurysms: Systematic Review and Meta-Analysis.

OBJECTIVE: The optimal treatment modality for saccular aneurysms of the posterior inferior cerebellar artery (PICA) remains unclear. A previous meta-analysis on the topic included a heterogenous study population, limiting the conclusions that can be drawn from its results. The aim of this study was to perform a systematic review and meta-analysis to compare outcomes of microsurgical and endovascular treatment (EVT) of these aneurysms. METHODS: A search of 4 online databases was performed for studies describing the management of saccular PICA aneurysms. The primary outcome was complete aneurysm occlusion. Data were also collected on neurologic outcomes, cranial nerve palsies, and requirement for re-treatment. A random effects model was used for calculation of pooled proportions. Our protocol was registered with PROSPERO (CRD42021232784). RESULTS: A total of 17 studies were included in the final analysis, reporting the treatment outcomes of 455 aneurysms, with a mean follow-up of 20 months. The pooled occlusion rates were 94.8% (95% confidence interval [CI] 90.6%-97.8%) for surgical treatment and 69.1% (95% CI 55.0%-81.7%) for EVT. Pooled rates of good neurologic outcome (modified Rankin scale score ≤2, Glasgow Outcome Scale score ≥4) at last follow-up were 78.1% (95% CI 67.4%-87.1%) for surgery and 77.6% (95% CI 67.9%-86.0%) for EVT. CONCLUSIONS: This meta-analysis demonstrates that in the treatment of saccular PICA aneurysms, microsurgical clipping results in superior angiographic outcomes, similar functional outcomes, but higher rates of lower cranial nerve palsy compared with EVT. Further studies are required to assess the duration and severity of cranial nerve palsies following surgical treatment, and long-term aneurysm occlusion and the requirement for re-intervention following EVT.

Multiple Meningiomas as a Criterion for the Diagnosis of Neurofibromatosis Type 2 and Other Tumor Predisposition Syndromes.

BACKGROUND: Bilateral vestibular schwannomas (VS) are pathognomonic of neurofibromatosis type 2 (NF2), but the diagnostic criteria also include unilateral VS (UVS) in combination with multiple meningiomas (MM) and other schwannomas, as well as MM without VS. OBJECTIVE: To investigate the diagnostic value of these criteria and establish the presence of other genetic conditions in patients presenting in this manner. METHODS: The Manchester International NF2 database was accessed to obtain information on patients presenting with a UVS and MM or ≥2 nonintradermal schwannomas (NIDS). We gathered data on patients diagnosed with NF2 due to MM without VS and on patients presenting with MM without meeting NF2 criteria. Analysis was performed for pathogenic variants (PVs) in NF2, SMARCE1, SMARCB1, and LZTR1. RESULTS: A total of 31 of 131 patients presenting with a UVS and MM had a nonrefuted diagnosis of NF2 after molecular studies, in comparison with 85 of 96 patients presenting with UVS and ≥2 NIDS (P ≤ .00001). Fifty percent of patients presenting with a UVS and ≥2 NIDS with NF2 developed bilateral VS, compared with only 26% of those who presented with a UVS and MM (P = .0046). In total, 11 of 152 patients presenting with MM without fulfilling NF2 criteria were found to have a PV in SMARCE1, and 7 of 152 were confirmed to have mosaic NF2. CONCLUSION: Patients presenting with UVS and MM are significantly more likely to have a nonrefuted diagnosis of NF2 than patients presenting with UVS and ≥2 NIDS, but significantly less likely to develop bilateral VS. Seven percent of those presenting with MM without meeting NF2 criteria had PV in SMARCE1, and 5% had mosaic NF2.

Translabyrinthine resection of NF2 associated vestibular schwannoma with cochlear implant insertion.

The authors present the case of a 24-year-old female with neurofibromatosis type 2. Growth of the left vestibular schwannoma and progressive hearing loss prompted the decision to proceed to translabyrinthine resection with cochlear nerve preservation and cochlear implant insertion. Complete resection with preservation of the facial and cochlear nerves was achieved. The patient had grade 1 facial function and was discharged on postoperative day 4 following suturing of a minor CSF leak. This case highlights the feasibility of cochlear nerve preservation and cochlear implant insertion in appropriately selected patients, offering a combination of effective tumor control and hearing rehabilitation. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21122.

Methods of Skull Base Repair Following Endoscopic Endonasal Tumor Resection: A Review.

Following the introduction of fully endoscopic techniques for the resection of pituitary tumors, there was a rapid expansion of the indications for endonasal endoscopic surgery to include extrasellar tumors of the skull base. These techniques offer significant advantages over traditional open surgical approaches to the skull base, including improved tumor resection, and better post-operative neurological outcomes. Following their introduction, however, the initial rate of post-operative CSF leak was unacceptably high. Post-operative CSF leak following skull base surgery is a major source of morbidity, and can lead to the development of life-threatening intracranial infection. The use of vascularized naso-septal flaps transformed the management of these patients, significantly reducing the rate of post-operative CSF leak and increasing the number of patients that could benefit from this less invasive treatment modality. Adequate repair of iatrogenic defects in the skull base is of crucial importance for patients with skull base tumors, as the development of a post-operative CSF leak, and the associated complications can significantly delay the administration of the adjunctive oncological therapies these patients require. In this review, we provide an overview of the latest evidence regarding skull base reconstruction following endoscopic skull base surgery, and describe the skull base repair technique in use at our institution.