Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 2: The Translabyrinthine Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 2, we present a codified operative workflow for the translabyrinthine approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Seventeen consultant skull base surgeons (nine neurosurgeons and eight ENT [ear, nose, and throat]) with median of 13.9 years of experience (interquartile range: 18.1 years) of independent practice participated. There was a 100% response rate across both the Delphi rounds. The translabyrinthine approach had the following five phases and 57 unique steps: Phase 1, approach and exposure; Phase 2, mastoidectomy; Phase 3, internal auditory canal and dural opening; Phase 4, tumor debulking and excision; and Phase 5, closure. Conclusion We present Part 2 of a national, multicenter, consensus-derived, codified operative workflow for the translabyrinthine approach to vestibular schwannomas. The five phases contain the operative, steps, instruments, technique errors, and event errors. The codified translabyrinthine approach presented in this manuscript can serve as foundational research for future work, such as the application of artificial intelligence to vestibular schwannoma resection and comparative surgical research.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 1: The Retrosigmoid Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 1, we present a codified operative workflow for the retrosigmoid approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus, was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Eighteen consultant skull base surgeons (10 neurosurgeons and 8 ENT [ear, nose, and throat]) with median 17.9 years of experience (interquartile range: 17.5 years) of independent practice participated. There was a 100% response rate across both Delphi's rounds. The operative workflow for the retrosigmoid approach contained three phases and 40 unique steps as follows: phase 1, approach and exposure; phase 2, tumor debulking and excision; phase 3, closure. For the retrosigmoid approach, technique, and event error for each operative step was also described. Conclusion We present Part 1 of a national, multicenter, consensus-derived, codified operative workflow for the retrosigmoid approach to vestibular schwannomas that encompasses phases, steps, instruments, technique errors, and event errors. The codified retrosigmoid approach presented in this manuscript can serve as foundational research for future work, such as operative workflow analysis or neurosurgical simulation and education.

Feasibility of comparing medical management and surgery (with neurosurgery or stereotactic radiosurgery) with medical management alone in people with symptomatic brain cavernoma - protocol for the Cavernomas: A Randomised Effectiveness (CARE) pilot trial.

INTRODUCTION: The top research priority for cavernoma, identified by a James Lind Alliance Priority setting partnership was 'Does treatment (with neurosurgery or stereotactic radiosurgery) or no treatment improve outcome for people diagnosed with a cavernoma?' This pilot randomised controlled trial (RCT) aims to determine the feasibility of answering this question in a main phase RCT. METHODS AND ANALYSIS: We will perform a pilot phase, parallel group, pragmatic RCT involving approximately 60 children or adults with mental capacity, resident in the UK or Ireland, with an unresected symptomatic brain cavernoma. Participants will be randomised by web-based randomisation 1:1 to treatment with medical management and with surgery (neurosurgery or stereotactic radiosurgery) versus medical management alone, stratified by prerandomisation preference for type of surgery. In addition to 13 feasibility outcomes, the primary clinical outcome is symptomatic intracranial haemorrhage or new persistent/progressive focal neurological deficit measured at 6 monthly intervals. An integrated QuinteT Recruitment Intervention (QRI) evaluates screening logs, audio recordings of recruitment discussions, and interviews with recruiters and patients/parents/carers to identify and address barriers to participation. A Patient Advisory Group has codesigned the study and will oversee its progress. ETHICS AND DISSEMINATION: This study was approved by the Yorkshire and The Humber-Leeds East Research Ethics Committee (21/YH/0046). We will submit manuscripts to peer-reviewed journals, describing the findings of the QRI and the Cavernomas: A Randomised Evaluation (CARE) pilot trial. We will present at national specialty meetings. We will disseminate a plain English summary of the findings of the CARE pilot trial to participants and public audiences with input from, and acknowledgement of, the Patient Advisory Group. TRIAL REGISTRATION NUMBER: ISRCTN41647111.

Risk of Aneurysm Rupture (ROAR) study: protocol for a long-term, longitudinal, UK multicentre study of unruptured intracranial aneurysms.

INTRODUCTION: Unruptured intracranial aneurysms (UIA) are common in the adult population, but only a relatively small proportion will rupture. It is therefore essential to have accurate estimates of rupture risk to target treatment towards those who stand to benefit and avoid exposing patients to the risks of unnecessary treatment. The best available UIA natural history data are the PHASES study. However, this has never been validated and given the known heterogeneity in the populations, methods and biases of the constituent studies, there is a need to do so. There are also many potential predictors not considered in PHASES that require evaluation, and the estimated rupture risk is largely based on short-term follow-up (mostly 1 year). The aims of this study are to: (1) test the accuracy of PHASES in a UK population, (2) evaluate additional predictors of rupture and (3) assess long-term UIA rupture rates. METHODS AND ANALYSIS: The Risk of Aneurysm Rupture study is a longitudinal multicentre study that will identify patients with known UIA seen in neurosurgery units. Patients will have baseline demographics and aneurysm characteristics collected by their neurosurgery unit and then a single aggregated national cohort will be linked to databases of hospital admissions and deaths to identify all patients who may have subsequently suffered a subarachnoid haemorrhage. All matched admissions and deaths will be checked against medical records to confirm the diagnosis of aneurysmal subarachnoid haemorrhage. The target sample size is 20 000 patients. The primary outcome will be aneurysm rupture resulting in hospital admission or death. Cox regression models will be built to test each of the study's aims. ETHICS AND DISSEMINATION: Ethical approval has been given by South Central Hampshire A Research Ethics Committee (21SC0064) and Confidentiality Advisory Group support (21CAG0033) provided under Section 251 of the NHS Act 2006. The results will be disseminated in peer-reviewed journals. TRIAL REGISTRATION NUMBER: ISRCTN17658526.

A survey of the radiological follow-up of unruptured intracranial aneurysms in the United Kingdom.

OBJECTIVE: Unruptured intracranial aneurysms (UIA) are common. For many the treatment risks outweigh their risk of subarachnoid haemorrhage and patients undergo surveillance imaging. There is little data to inform if and how to monitor UIAs resulting in widely varying practices. This study aimed to determine the current practice of unruptured UIA surveillance in the United Kingdom. METHODS: A questionnaire was designed to address the themes of surveillance protocols for UIA including when surveillance is initiated, how frequently it is performed, and when it is terminated. Additionally, how aneurysm growth is managed and how clinically meaningful growth is defined were explored. The questionnaire was distributed to members of the British Neurovascular Group using probability-based cluster and non-probability purposive sampling methods. RESULTS: Responses were received from 30 of the 30 (100.0%) adult neurosurgical units in the United Kingdom of which 27 (90.0%) routinely perform surveillance for aneurysm growth. Only four units had a unit policy. The mean patient age up to which a unit would initiate follow-up of a low-risk UIA was 65.4 ± 9.0 years. The time points at which imaging is performed varied widely. There was an even split between whether units use a fixed duration of follow-up or an age threshold for terminating surveillance. Forty percent of units will follow-up patients more than 5 years from diagnosis. The magnitude in the change in size that was felt to constitute growth ranged from 1 to 3mm. No units routinely used vessel wall imaging although 27 had access to 3T MRI capable of performing it. CONCLUSIONS: There is marked heterogeneity in surveillance practices between units in the United Kingdom. This study will help units better understand their practice relative to their peers and provide a framework forplanning further research on aneurysm growth.

Skull Base Neurosurgery Eponyms: A Review of Notable Anatomical Landmarks.

Eponyms highlight the contributions made to medicine over the years, and celebrate individuals for their work involving diseases, pathologies, and anatomical landmarks. We have compiled an in-depth report of eponyms used in skull base neurosurgery, as well as the historical contexts of the personalities behind the names. A literature search identified 36 eponyms of the bones, foramina and ligaments of the skull base named after anatomists and physician-scientists. The 36 eponymous structures pinpointed include Arnold's canal, the foramen of Arnold, Bill's bar, Bertin's bones, Civinini's canal, Civinini's ligament, Civinini's process, sinodural angle of Citelli, Clivus of Blumenbach, Dorello's canal, the Eustachian tube, the eponymous cavernous sinus triangles of Parkinson, Kawase, Mullan, Dolenc, Glasscock and Hakuba, the Fallopian canal, the Glasserian fissure, Gruber's ligament, Haller cells, the spine of Henle, Highmore's antrum, the foramen of Huschke, Hyrtl's fissure, the Ingrassia process, Jacobson's canal, the MacEwen triangle, Meckel's cave, the Onodi air cell, the Pacchionian foramen, Fossa of Rosenmuller, the foramen of Vesalius, the Vidian canal, Trautman's triangle and the annular tendon of Zinn. Knowledge of the relevant eponyms enables succinct descriptions of important skull base structures, provides an understanding of associated clinical implications, and reminds us of the vast history of contributions to neurosurgery made by prominent figures in the field.

Evaluating growth trends of residual sporadic vestibular schwannomas: a systematic review and meta-analysis.

BACKGROUND: Gross total resection remains the gold-standard approach for vestibular schwannomas (VS) when surgery is indicated. In select cases, incomplete resection (IR) becomes a desired alternative to preserve the facial nerve function and the patient's quality of life. While a lot of earlier studies described incompletely resected sporadic VSs as dormant, more recent studies reported a higher growth rate following IR, therefore an evaluation of the residual VS growth rates could have important implications for the follow-up treatment protocols and provide relevant information for neurosurgeons, neuro-otologists, neuropathologists, and radiologists. Although prognostic factors predicting preoperative VS growth have been previously investigated, these factors have not been investigated following IR. Our review aims to examine the growth rate of residual sporadic VS following IR and to examine variables associated with the regrowth of residual VS. METHODS: The review was conducted in accordance with the PRISMA guidelines. Six databases (MEDLINE (Ovid), Embase (Ovid), CINAHL Plus (EBSCO), Cochrane Central Register of Controlled Trials (CENTRAL), WHO International Clinical Trials Registry Platform and UK Clinical Trials Gateway (WHO ICTRP) were searched. Full-text articles analysing growth rates in at least ten patients who had residual VS after IR were assessed. We conducted a meta-analysis using a random-effects model via RevMan. RESULTS: 14 studies totalling 849 patients were included in the analysis. The mean planimetric growth rate was 1.57 mm/year (range 0.16-3.81 mm/year). The mean volumetric growth rate was 281.725 mm3/year (range 17.9-530.0 mm3/year). Age, sex, pre-operative tumour size/volume, cystic tumour sub-type, MIB-1 index, and intracanalicular tumour location were not associated with residual growth. Residual tumour size/volume was statistically significant to growth (OR = 0.65, 95% CI 0.47-0.90, p = 0.01). Radiological re-growth occurred in an average of 26.6% of cases (range 0-54.5%). CONCLUSION: From our analysis, only the residual tumour volume/size was associated with residual VS growth. Therefore, close postoperative surveillance for the first year, followed by an annual MRI scan for at least 5 years, and subsequently extended interval surveillance remains of utmost importance to monitor disease progression and provide timely surgical and adjuvant interventions. Our study shows that future work should be aimed at molecular and histological characteristics of residual VSs to aid prognostic understanding of growth.

Can further subdivision of the Raymond-Roy classification of intracranial aneurysms be useful in predicting recurrence and need for future retreatment following endovascular coiling?

Background: The Raymond-Roy classification has been the standard for neck recurrences following endovascular coiling with three grades. Several modified classification systems with subdivisions have been reported in literature but it is unclear whether this adds value in predicting recurrence or retreatment. Our aim is to assess if these subdivisions aid in predicting recurrence and need for retreatment. Methods: A retrospective review of all patients undergoing endovascular coiling between 2013 and 2014. Patients requiring stent assistance or other embolization devices were excluded from the study. The neck residue was graded at time of coiling on the cerebral angiogram and subsequent 6, 24, and 60 months MRA. Correlation between grade at coiling and follow-up with need for subsequent retreatment was assessed. Results: Overall, 17/200 (8.5%) cases required retreatment within 5 years of initial coiling. 4/130 (3.1%) required retreatment within 5 years with initial Grade 0 at coiling, 6/24 cases (25%) of those Grade 2a, 4/20 cases (20%) Grade 2b, 3/8 (38%) Grade 3, and none of those with Grade 1. Large aneurysms ≥11 mm had an increased risk of aneurysm recurrence and retreatment. About 9.7% of ruptured aneurysms required retreatment versus 4.4% for unruptured. About 55% of carotid ophthalmic aneurysms were retreated. Conclusion: Although the modified classification system was significantly predictive of progressive recurrence and need for retreatment, no significant difference between the subdivisions of Grade 2 was observed. Similar predictive value was seen when using the Raymond-Roy classification compared to the new modified, limiting the usefulness of the new system in clinical practice.

Cavernous sinus haemangioma: systematic review and pooled analysis relating to a rare skull base pathology.

Cavernous sinus haemangiomas (CSHs) are rare malformations of the microcirculation arising from the cavernous sinus. A systematic review and pooled data analysis of the associated clinical features, diagnostic modalities, management, and outcomes for CSHs was done. In total, 68 articles (338 cases) were eligible for analysis based on our selection criteria. The primary outcome measures were the occurrence of (i) and (ii) symptom resolution/improvement. Categorical outcome variables were assessed by binary logistic regression at 5% significance level. With headaches (39.9%) and diplopia (36.5%) as the most common presenting symptoms reported, dynamic contrast-enhanced MRI was the most commonly used diagnostic modality and was the most definitive pre-treatment imaging modality for diagnosing CSH with a sensitivity of 89.5%. The majority of CSHs were managed with radiosurgery (47.9% of cases), 37.9% by surgical resection alone, and 14.2% by a combination of both. Compared to patients that were treated with surgical resection only, those treated solely with radiosurgery had a 100% decrease in the odds of developing post-treatment complications (adjusted OR: 0.00, 95% CI: 0.00-0.002, p < 0.001), with a 5.03 times greater odds of symptom resolution/improvement (adjusted OR: 5.03, 95% CI: 1.89-13.4, p = 0.001). Patients that underwent combined therapy had a 79% reduction in risk of developing post-treatment complications (adjusted OR: 0.21, 95% CI: 0.06-0.68, p = 0.01), with no statistically significant difference in the odds of symptom resolution/improvement, compared to those that had surgery only. In conclusion, radiosurgery offered the best outcomes with regards to symptom resolution/improvement and post-treatment complications in patients with CSH.

Revising Fourier-transform infrared (FT-IR) and Raman spectroscopy towards brain cancer detection.

Fourier-transform infrared (FT-IR) and Raman spectroscopy are being widely applied as sensor-based techniques in oncology, particularly in the diagnosis of brain cancers and their subtypes. Overtime, these techniques have become more sensitive; and, accuracies of over 90% have been observed in several studies. This is indication of their potential for clinical implementation. Herein, we present a mini-review by revisiting some fundamentals of FT-IR and Raman spectroscopy along with their applications towards brain cancer detection in the literature.

Deep Learning in the Management of Intracranial Aneurysms and Cerebrovascular Diseases: A Review of the Current Literature.

Intracranial aneurysms are a common asymptomatic vascular pathology, the rupture of which is a devastating event with a significant risk of morbidity and mortality. Aneurysm detection and risk stratification before rupture events are, therefore, imperative to guide prophylactic measures. Artificial intelligence has shown great promise in the management pathway of aneurysms, through automated detection, the prediction of rupture risk, and outcome prediction after treatment. The complementary use of these programs, in addition to clinical practice, has demonstrated high diagnostic and prognostic accuracy, with the potential to improve patient outcomes. In the present review, we explored the role and limitations of deep learning, a subfield of artificial intelligence, in the aneurysm patient journey. We have also briefly summarized the application of deep learning models in automated detection and prediction in cerebral arteriovenous malformations and Moyamoya disease.

Raman hyperspectral imaging coupled to three-dimensional discriminant analysis: Classification of meningiomas brain tumour grades.

Meningiomas remains a clinical dilemma. They are the commonest "benign" types of brain tumours and, although being typically benign, they are divided into three WHO grades categories (I, II and III) which are associated with the tumour growth rate and likelihood of recurrence. Recurrence depends on extend of surgery as well as histopathological diagnosis. There is a marked variation amongst surgeons in the follow-up arrangements for their patients even within the same unit which has a significant clinical, and financial implication. Knowing the tumour grade rapidly is an important factor to predict surgical outcomes and adequate patient treatment. Clinical follow up sometimes is haphazard and not based on clear evidence. Spectrochemical techniques are a powerful tool for cancer diagnostics. Raman hyperspectral imaging is able to generate spatially-distributed spectrochemical signatures with great sensitivity. Using this technique, 95 brain tissue samples (66 meningiomas WHO grade I, 24 meningiomas WHO grade II and 5 meningiomas that reoccurred) were analysed in order to discriminate grade I and grade II samples. Newly-developed three-dimensional discriminant analysis algorithms were used to process the hyperspectral imaging data in a 3D fashion. Three-dimensional principal component analysis quadratic discriminant analysis (3D-PCA-QDA) was able to distinguish grade I and grade II meningioma samples with 96% test accuracy (100% sensitivity and 95% specificity). This technique is here shown to be a high-throughput, reagent-free, non-destructive, and can give accurate predictive information regarding the meningioma tumour grade, hence, having enormous clinical potential with regards to being developed for intra-operative real-time assessment of disease.

The Role of Endoscopy in the Resection of Sporadic Vestibular Schwannomas: A Systematic Review of Surgical Outcomes.

BACKGROUND: Vestibular schwannomas (VS) are benign intracranial tumors originating from the vestibular division of the eighth cranial nerve. Treatment options include microsurgery, radiotherapy, and surveillance. Endoscopy is becoming more widely used as an adjunct in skull base surgery and may influence outcomes in surgically managed VS. METHODS: A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies involving endoscope-assisted and fully endoscopic procedures for sporadic VS resection were identified. Facial nerve function, hearing preservation, extent of resection, and complications were analyzed. RESULTS: Thirty-one studies were included (27 endoscope-assisted, four fully endoscopic). Subgroup analyses were performed to assess outcomes according to tumor size and surgical approach. Overall, endoscopic facial nerve preservation rates were comparable to microsurgical treatment. A subgroup analysis suggested that functional facial nerve preservation rates may be higher when endoscopic assistance is used for smaller (Koos I-II) tumors using the retrosigmoid or translabyrinthine approach. The gross total resection rate for small tumors was higher in retrosigmoid ES-assisted microsurgery (96.2%) compared to rates in the literature for the standard, open retrosigmoid approach. Hearing outcomes were more variable and were under-reported. CONCLUSIONS: Current data suggest that ES-assisted resection of sporadic VS is not inferior to microsurgical resection with respect to facial nerve outcomes and extent of resection. However, some ES series report poor hearing outcomes, which are under-reported in the literature. Further prospective studies are required to ascertain if endoscopic assistance can improve outcomes for VS resection, particularly for smaller (Koos I-II) tumors.

Neurosurgery activity levels in the United Kingdom and republic of Ireland during the first wave of the covid-19 pandemic - a retrospective cross-sectional cohort study.

The impact of Covid-19 on surgical patients worldwide has been substantial. In the United Kingdom (UK) and the Republic of Ireland (RoI), the first wave of the pandemic occurred in March 2020. The aims of this study were to: (1) evaluate the volume of neurosurgical operative activity levels, Covid-19 infection rate and mortality rate in April 2020 with a retrospective cross-sectional cohort study conducted across 16 UK and RoI neurosurgical centres, and (2) compare patient outcomes in a single institution in April-June 2020 with a comparative cohort in 2019. Across the UK and RoI, 818 patients were included. There were 594 emergency and 224 elective operations. The incidence rate of Covid-19 infection was 2.6% (21/818). The overall mortality rate in patients with a Covid-19 infection was 28.6% (6/21). In the single centre cohort analysis, an overall reduction in neurosurgical operative activity by 65% was observed between 2020 (n = 304) and 2019 (n = 868). The current and future impact on UK neurosurgical operative activity has implications for service delivery and neurosurgical training.

Identifying Factors Associated with the Growth of Vestibular Schwannomas: A Systematic Review.

BACKGROUND: Surveillance imaging is a valid management option for selected vestibular schwannomas (VS). An ideal protocol for radiologic monitoring would highlight growth-related risk factors and tailor management accordingly. This study aims to identify variables associated with the growth of sporadic VS to enhance surveillance imaging, enable early intervention, and optimize outcomes. METHODS: The review was conducted according to the PRISMA guidelines. A systematic review of 5 databases (PubMed, Ovid, Cochrane Library, Web of Science, and Google Scholar) was performed to identify negative and positive growth predictors of sporadic vestibular schwannomas. The search was limited to studies reported between January 2015 and January 2020. We conducted an individual patient data meta-analysis using a 1-stage multivariate mixed-effect logistic regression model. RESULTS: A total of 437 studies were identified, of which 25 met our criteria for full-text analysis. Articles that measured VS with comparable methods were determined eligible for meta-analysis inclusion. The selected articles were highly heterogeneous in their use of grading scales and assessment of tumor size. Our review showed that size at diagnosis (odds ratio, 1.15; 95% confidence interval, 1.11-1.18; P < 0.0001) and intracanalicular localization (odds ratio, 0.49; 95% confidence interval, 0.26-0.90; P = 0.023) were associated with VS growth. CONCLUSIONS: The factors most frequently reported as being associated with growth within the literature were size of VS at diagnosis and localization of an intracanalicular component. Greater attention should be placed on these criteria within the surveillance imaging algorithm for VS.

Radiological surveillance of small unruptured intracranial aneurysms: a systematic review, meta-analysis, and meta-regression of 8428 aneurysms.

With the widespread use of imaging techniques, the possibility that an asymptomatic unruptured intracranial aneurysm (UIA) is detected has increased significantly. There is no established consensus regarding follow-up, duration, and frequency of such imaging surveillance. The objectives of this study include assessing the growth rate and rupture risk of small (less than 7mm) UIAs, identifying associated risk factors and providing an aneurysm surveillance protocol in appropriately selected patients. Systematic searches of Medline, Embase, and Cochrane Central were undertaken from database inception to March 2020 for published studies reporting the growth and rupture risks of small UIAs. Twenty-one studies reporting 8428 small UIAs were included in our meta-analysis. The pooled mean age was 61 years (95% CI: 55-67). The mean follow-up period for growth and rupture ranged from 11 to 108 months, with the pooled mean follow-up period across 14 studies being 42 months (95% CI: 33-51). Pooled overall growth rate was 6.0% (95% CI: 3.8-8.7). Pooled growth rates for aneurysms < 5mm and < 3 mm were 5.2% (95% CI: 3.0-7.9) and 0.8% (95% CI: 0.0-6.1), respectively. Pooled overall rupture rate was 0.4% (95% CI: 0.2-0.7). From the meta-regression analysis, having multiple aneurysms, smoking, hypertension, and personal history of SAH did not significantly predict growth, and a personal history of SAH, smoking, hypertension, and multiple aneurysms were not statistically significant predictors of rupture. Our findings suggest that small UIAs have low growth and rupture rates and very small UIAs have little or no risk for rupture. In the setting of incidental small UIAs, patients with multiple and/or posterior circulation aneurysms require more regular radiological monitoring.

Intracranial Dural Arteriovenous Fistula Presenting With Bulbar and Myelopathic Features: Case Report and Literature Review.

Intracranial dural arteriovenous fistulas are defined as pathological anastomoses between meningeal arteries and dural venous sinuses or cortical veins. Rarely, they could drain into the venous system in and around the craniocervical junction and cause myelopathy and/or bulbar features. Due to the clinical and radiological features, prompt diagnosis poses a challenge, as there are several neurological differential diagnoses. Several mechanisms for the presentation have been considered, such as venous hypertension, direct compression by enlarged veins, and ischaemia due to infarcts or due to arterial steal. Digital subtraction angiography (DSA) is the gold-standard diagnosis for dural arteriovenous fistulas, where the visualisation of feeding arteries and the characterisation of venous drainage allows diagnosis and grading. Treatment options include conservative management, invasive (microsurgery) or minimally invasive options (transarterial or transvenous embolisation). We present a 32-year-old male who presented with myelopathy and bulbar features. Angiography demonstrated an intracranial dural arteriovenous fistula, which was successfully treated surgically.

De Novo Cavernoma Formation in a Patient With Cowden Syndrome and Lhermitte-Duclos Disease.

BACKGROUND: Cowden syndrome (CS) is an autosomal dominant genodermatosis with a predisposition for the development of multiple cancers, benign hamartomas, and extracranial vascular malformations. Rarely, intracranial lesions like meningiomas and vascular malformations can also be present with CS. These vascular malformations include developmental venous anomalies, arteriovenous fistulae and cavernomas. Most cases of cavernomas are thought to be congenital, although in recent literature they have been shown to occur de novo with other conditions (e.g., other vascular malformations, trauma, postcranial surgery, viral infection, and genetic disorders). CASE DESCRIPTION: We present a 29-year-old woman who was diagnosed with Lhermitte-Duclos disease after episodes of persistent generalized headaches. She underwent a foramen magnum decompression and was subsequently diagnosed with CS. Ten years, later she was also diagnosed with 2 cerebral cavernomas that were not present on her prior monitoring scans. CONCLUSIONS: We present a case of a patient with CS and LDD who had de novo cavernoma development several years after the initial diagnosis, as well as a review of the literature. We highlight the need of surveillance neuroimaging for patients with CS, as there is the risk of new development of vascular abnormalities (particularly cavernomas).

Multiple cerebral cavernous malformations in association with a Dubowitz-like syndrome.

Cerebral cavernous malformations (CCMs) are proliferative sinusoidal vascular lesions and are the most common vascular malformations of the brain. They can occur sporadically or secondary to an underlying genetic predisposition where multiple lesions are commonly seen. Dubowitz syndrome is a clinically-diagnosed rare genetic disorder with an unknown molecular basis. An association between these conditions has not been reported previously. A 30-year-old woman with a Dubowitz-like syndrome presented with acute left leg weakness, gait ataxia and transient loss of consciousness. Imaging revealed five CCMs with recent hemorrhage in relation to one lesion in the left middle cerebellar peduncle. A recurrent hemorrhage from the same lesion occurred ten weeks later and she underwent microsurgical excision of this malformation. Genetic analysis revealed an unbalanced chromosomal rearrangement involving partial deletion of chromosome 7q21, the locus of the CCM1/KRIT1 gene known to be associated with familial CCMs. This is the first description of CCMs in association with the Dubowitz phenotype. The genetic basis of Dubowitz syndrome may be heterogeneous but, for the first time, overlap is demonstrated between this condition and multiple CCMs, with a possible common genetic etiology. Knowledge of this association may be of help in the management of acute neurological presentations in Dubowitz-like syndromes. Keywords: Hemangioma, Cavernous, Central nervous system, Dubowitz syndrome, Genetics.

Spectrochemical differentiation of meningioma tumours based on attenuated total reflection Fourier-transform infrared (ATR-FTIR) spectroscopy.

Meningiomas are the commonest types of tumours in the central nervous system (CNS). It is a benign type of tumour divided into three WHO grades (I, II and III) associated with tumour growth rate and likelihood of recurrence, where surgical outcomes and patient treatments are dependent on the meningioma grade and histological subtype. The development of alternative approaches based on attenuated total reflection Fourier-transform infrared (ATR-FTIR) spectroscopy could aid meningioma grade determination and its biospectrochemical profiling in an automated fashion. Herein, ATR-FTIR in combination with chemometric techniques is employed to distinguish grade I, grade II and grade I meningiomas that re-occurred. Ninety-nine patients were investigated in this study where their formalin-fixed paraffin-embedded (FFPE) brain tissue samples were analysed by ATR-FTIR spectroscopy. Subsequent classification was performed via principal component analysis plus linear discriminant analysis (PCA-LDA) and partial least squares plus discriminant analysis (PLS-DA). PLS-DA gave the best results where grade I and grade II meningiomas were discriminated with 79% accuracy, 80% sensitivity and 73% specificity, while grade I versus grade I recurrence and grade II versus grade I recurrence were discriminated with 94% accuracy (94% sensitivity and specificity) and 97% accuracy (97% sensitivity and 100% specificity), respectively. Several wavenumbers were identified as possible biomarkers towards tumour differentiation. The majority of these were associated with lipids, protein, DNA/RNA and carbohydrate alterations. These findings demonstrate the potential of ATR-FTIR spectroscopy towards meningioma grade discrimination as a fast, low-cost, non-destructive and sensitive tool for clinical settings. Graphical abstract Attenuated total reflection Fourier-transform infrared (ATR-FTIR) spectroscopy was used to discriminate meningioma WHO grade I, grade II and grade I recurrence tumours.