A rare case of multifocal craniospinal leptomeningeal melanocytoma: A case report and scoping review.

Introduction: Leptomeningeal melanocytomas are rare tumours originating from neural crest derived melanocytes. They are usually solitary and presentation with multifocal meningeal melanocytoma is very rare and indicative of potentially more aggressive behaviour. This case report and scoping review sought to evaluate the presentation, and key radiological features that can help differentiate multifocal meningeal melanocytoma from other differentials and provide a discussion of the key management and prognostic points once these tumours are diagnosed. Case presentation: A 26 year old male presented with neck pain radiating to both shoulders and subjective weakness in left shoulder movement. MRI demonstrated a large enhancing C2-C3 intradural-extramedullary lesion with further lesions at the T7/T8 level, left cerebellopontine angle and midline suprachiasmatic region. Whilst the imaging appearances were initially thought be indicative of a phacomatosis such as NF2-related schwannomatosis, surgical excision of the cervical tumour confirmed a melanocytic tumour of leptomeningeal origin, consistent with multifocal meningeal melanocytoma. Patient made a good post-operative recovery and remains under half yearly radiological surveillance, with repeat MRI 6 months after surgery demonstrating subtle growth of the untreated intracranial and spinal lesions. Literature review and conclusions: This is the first description, to our knowledge, of a multifocal meningeal melanocytoma associated with both cerebellopontine angle and suprasellar lesions. This case and included scoping review highlight the need to consider this rare diagnosis whenever multifocal craniospinal lesions are encountered, and the need to consider aggressive management through surgical resection and adjuvant craniospinal radiotherapy once these tumours are diagnosed.

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.

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.

Pilot application of Lecture-Panel-Discussion Model (LPDM) in global collaborative neurosurgical education: a novel training paradigm innovated by the Swedish African Neurosurgery Collaboration.

BACKGROUND: Disruptions in global surgery educational routines by the COVID-19 pandemic have elicited demands for alternative formats for rendering qualitative neurosurgical education. This study presents application of a novel model of online neurosurgical course, the Lecture-Panel-Discussion Model (LPDM). METHODS: This is a cross-sectional survey of participants who attended the Swedish African Neurosurgery Collaboration (SANC)-100A course. Participants evaluated the course through an online self-administered questionnaire using a 5-point Likert scale ranging from very poor-1, poor-2, average (fair)-3, good-4, to excellent-5. SANC-100A comprises a tripod of Lectures, Panel review, and interactive case Discussion. This model (LPDM) was innovated by SANC and applied at the Enugu International Neurosurgery course in February 2021. RESULTS: There were 71 attendees, 19 were course faculty, while 52 were participants. Thirty-five attended from Nigeria, 11 from Sweden, 3 from Malawi, 2 from Senegal, and 1 from the UK. Among 44 participants who completed the questionnaire, there were 9 fellows and 35 residents. The overall median course Likert rating was 4.65 ± 0.1. The median overall rating for course events was similar between day 1 (Likert score = 4.45) and day 2 (Likert score = 4.55), U = 55, Z score = 1.10, P = 0.27. The median rating for lectures was 4.50 ± 0.2 and varied from 4.40 on day 1 to 4.55 on day 2. The median rating for panel review was 4.60 ± 0.1 and varied from 4.55 on day 1 to 4.65 on day 2. Interactive case discussions were rated 4.80 on both course days. There was a significant variability in the rating profiles of the course tripod: U = 24.5, P = 0.03. Fifty-one (98%) participants believe LPDM was COVID-19-compliant, while 90% believe the course was beneficial to training and practice. CONCLUSION: Initial application of LPDM is rewarded with both high acceptance and high rating among participants.

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.

Development and Growth of Intracranial Meningiomas in Transgender Women Taking Cyproterone Acetate as Gender-Affirming Progestogen Therapy: A Systematic Review.

Background: Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used in high doses as gender-affirming progestogen therapy in transgender women. An association between high-dose CPA and the development and growth of intracranial meningioma, including case reports in transgender women, has been described. This systematic review summarizes these cases at the patient level and discusses their management. Methods: This systematic review was registered with PROSPERO (CRD42020191965). A detailed search of the PubMed, EMBASE, and Web of Science electronic bibliographic databases was performed (inception-December 20, 2020). Two review authors independently completed screening, data extraction, and risk of bias assessment in duplicate. Results: Nine records were included describing (n=12) individual case reports and (n=35) intracranial meningiomas. The median age at presentation was 48 years (interquartile range [IQR], 43-55 years), most frequent daily CPA doses were 50 mg/day (n=5) and 100 mg/day (n=5), and the median duration of CPA use was 9.5 years (IQR, 6.5-17.5 years). Multiple meningiomas were common (n=7). For most cases (n=10), surgical resection was the initial preferred management strategy, but two were successfully managed by CPA cessation. Conclusions: Transgender women receiving high doses of CPA may be at increased risk of intracranial meningioma development and/or growth, although this remains a rare disease. For presumed CPA-associated meningioma, drug cessation appears to be an appropriate management strategy when surgery is not imminently required to manage raised intracranial pressure or prevent neurological deterioration. Given the importance of gender-affirming hormone therapy to transgender persons, a suitable alternative hormone regimen should be offered, although the use of CPA in both high doses and for prolonged periods of time is now in decline.

Gender-affirming hormone therapy associated with multiple meningiomas and atypical histology in a transgender woman.

We present a 69-year-old transgender woman who underwent gender-affirming surgery in 1998 and gender-affirming hormone therapy (cyproterone acetate (CPA) and estradiol) since this time. Following an MRI scan to investigate tremor in 2013, an incidental left anterior clinoid and right petrous meningioma were identified. Subtotal surgical resection was achieved for the anterior clinoid meningioma (WHO grade 1, meningothelial subtype). At follow-up in 2016, an olfactory groove meningioma and left greater wing of sphenoid meningioma were identified. By 2017, both tumours, along with the petrous meningioma, demonstrated significant growth. In 2018, clinical decline was evident and MRI demonstrated further tumour growth. Surgery was scheduled and the olfactory groove meningioma was completely resected (WHO grade 2, chordoid subtype). Hormones were stopped, after which regression of the petrous meningioma was observed. This case demonstrates an association between high-dose CPA and estradiol and the development, growth and regression of meningiomas in a transgender woman.

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.

CSF rhinorrhoea after endonasal intervention to the anterior skull base (CRANIAL): proposal for a prospective multicentre observational cohort study.

BACKGROUND: The endonasal transsphenoidal approach (TSA) has emerged as the preferred approach in order to treat pituitary adenoma and related sellar pathologies. The recently adopted expanded endonasal approach (EEA) has improved access to the ventral skull base whilst retaining the principles of minimally invasive surgery. Despite the advantages these approaches offer, cerebrospinal fluid (CSF) rhinorrhoea remains a common complication. There is currently a lack of comparative evidence to guide the best choice of skull base reconstruction, resulting in considerable heterogeneity of current practice. This study aims to determine: (1) the scope of the methods of skull base repair; and (2) the corresponding rates of postoperative CSF rhinorrhoea in contemporary neurosurgical practice in the UK and Ireland. METHODS: We will adopt a multicentre, prospective, observational cohort design. All neurosurgical units in the UK and Ireland performing the relevant surgeries (TSA and EEA) will be eligible to participate. Eligible cases will be prospectively recruited over 6 months with 6 months of postoperative follow-up. Data points collected will include: demographics, tumour characteristics, operative data), and postoperative outcomes. Primary outcomes include skull base repair technique and CSF rhinorrhoea (biochemically confirmed and/or requiring intervention) rates. Pooled data will be analysed using descriptive statistics. All skull base repair methods used and CSF leak rates for TSA and EEA will be compared against rates listed in the literature. ETHICS AND DISSEMINATION: Formal institutional ethical board review was not required owing to the nature of the study - this was confirmed with the Health Research Authority, UK. CONCLUSIONS: The need for this multicentre, prospective, observational study is highlighted by the relative paucity of literature and the resultant lack of consensus on the topic. It is hoped that the results will give insight into contemporary practice in the UK and Ireland and will inform future studies.

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.

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).

Cerebral Venous Sinus Thrombosis After Translabyrinthine Vestibular Schwannoma-A Prospective Study and Suggested Management Paradigm.

OBJECTIVE: To prospectively evaluate the incidence, risk factors and natural history of postoperative cerebral venous sinus thrombosis (pCVST) in translabyrinthine vestibular schwannoma microsurgical resection and propose a potential management paradigm. STUDY DESIGN: Prospective, single cohort, multicenter study. SETTING: State-wide academic tertiary referral centers. PATIENTS: Fifty-four consecutive patients who underwent translabyrinthine vestibular schwannoma resection. MAIN OUTCOME MEASURES: Incidence of pCVST on postoperative imaging on Day 7, Day 28, and 12 months postoperatively. Patients and tumor characteristics, risk factors, length of stay, intraoperative parameters, complications, and follow-up were analyzed. RESULTS: pCVST was demonstrated in 21 patients (38.9%) on postoperative imaging. All patients with pCVST were treated conservatively and remained asymptomatic in the immediate postoperative period and long-term follow-up. There were no instances of venous infarction, intracranial hemorrhage, or neurological deficits. A majority (61.1%) of pCVST recannalised on long-term follow up with conservative management. There was a statistical association with pCVST and surgery on the side of the non-dominant cerebral venous drainage (n = 17, 80.1%, p = 0.034). Patients with pCVST were significantly more likely to have a postoperative cerebrospinal fluid (CSF) leak (n = 5, 23.8%, p = 0.017). CONCLUSION: The incidence of pCVST following translabyrinthine vestibular schwannoma resection is much higher than previously recognized. pCVST is more likely to occur when surgery is performed on tumors situated on the side of non-dominant cerebral venous drainage. Despite the high prevalence of this iatrogenic phenomenon, all patients were asymptomatic and a majority resolved, thereby suggesting that conservative management is safe. Correlation between pCVST and increased incidence of CSF leak requires further investigation.

A Dual Approach for the Management of Complex Craniovertebral Junction Abnormalities: Endoscopic Endonasal Odontoidectomy and Posterior Decompression with Fusion.

BACKGROUND: Ventral brainstem compression secondary to complex craniovertebral junction abnormality is an infrequent cause of neurologic deterioration in pediatric patients. However, in cases of symptomatic, irreducible ventral compression, 360° decompression of the brainstem supported by posterior stabilization may provide the best opportunity for improvement in symptoms. More recently, the endoscopic endonasal corridor has been proposed as an alternative method of odontoidectomy associated with less morbidity. We report the largest single case series of pediatric patients using this dual-intervention surgical technique. The purpose of this study was to evaluate the surgical outcomes of pediatric patients who underwent posterior occipitocervical decompression and instrumentation followed by endoscopic endonasal odontoidectomy performed to relieve neurologic impingement involving the ventral brainstem and craniocervical junction. METHODS: Between January 2011 and February 2017, 7 patients underwent posterior instrumented fusion followed by endonasal endoscopic odontoidectomy at our unit. Standardized clinical and radiological parameters were assessed before and after surgery. A univariate analysis was performed to assess clinical and radiologic improvement after surgery. RESULTS: A total of 14 operations were performed on 7 pediatric patients. One patient had Ehlers-Danlos syndrome, 1 patient had a Chiari 1 malformation, and the remaining 5 patients had Chiari 1.5 malformations. Average extubation day was postoperative day 0.9. Average day of initiation of postoperative feeds was postoperative day 1.0. CONCLUSIONS: The combined endoscopic endonasal odontoidectomy and posterior decompression and fusion for complex craniovertebral compression is a safe and effective procedure that appears to be well tolerated in the pediatric population.

Endoscopic skull base neurosurgical practice in the United Kingdom.

Background: Endoscopic skull base surgery (ESBS) is a new subspecialty area that has become more popular over the past 20 years. It is fast evolving and the indications are getting increasingly diverse. The skill-sets also continue to significantly improve, hence, we, the authors aim to assess the current status of this unique sub-specialty within the United Kingdom. Objective: Our objectives included identifying the proportion of neurosurgeons within the United Kingdom who utilize endoscopic skull base approaches, the background training involved and how much of the overall neurosurgical workload is constituted by endoscopic skull base neurosurgery. There was also a focus on the technical nuances involved with ESBS. Materials and methods: 45 neurosurgeons were included in this study, and a 47% response rate (n = 21) was obtained. Results: 7 (33%) had training in ESBS during residency and 8 (38%) had some fellowship exposure to ESBS. Each respondent did an average of 1.9 endoscopic skull base courses prior to commencing their practice. The length of practice ranged from 3 to 15 years (mean - 7.2 years). Although most of the surgeons had mixed sub-specialty interests, the most commonly associated sub-specialty with ESBS was lateral skull base surgery (38%). Conclusion: In the United Kingdom, ESBS appears to be commonly combined with a lateral/open skull base neurosurgical practice. Most of the surgeons had their ESBS training after formal residency. Collaboration with ENT occurs more commonly for extended transphenoidal procedures.

Treatment strategies for craniopharyngiomas.

Craniopharyniomas arise from the sellar region and are particularly challenging because of their close proximity to critical neurovascular structures, including cranial nerves, brainstem, internal carotid arteries, posterior cerebral arteries, hypothalamus, and the pituitary gland. The tumors are benign on histology but can cause serious symptoms by compression of surrounding vital structures. While radical surgery and gross total resection (GTR) remains the first line treatment, choosing the optimal surgical approach is critical for the minimization of complications and postoperative morbidity. The extended endoscopic endonasal approach (EEEA) offers a direct route towards the sellar and suprasellar regions and has proven to be a safe and effective for GTR, with lower rates of complications and postoperative morbidity than open transcranial approaches. However, traditional transcranial approaches remain relevant in tumors with significant lateral extension. Radiotherapy can be considered in cases of subtotal resection (STR) or if surgery is not possible, although progressive growth of cysts with neurological deterioration has been demonstrated no infrequently. Newly developed chemotherapy for papillary craniopharygniomas might expand treatment options in the future.

Endoscopic endonasal resection of epidermoid cysts involving the ventral cranial base.

OBJECTIVEEpidermoid cysts (ECs) commonly extend to involve the ventral cisterns of the cranial base. When present, symptoms arise due to progressive mass effect on the brainstem and adjacent cranial nerves. Historically, a variety of open microsurgical approaches have been used for resection of ECs in this intricate region. In recent years, the endoscopic endonasal approach (EEA) has been proposed as an alternative corridor that avoids crossing the plane of the cranial nerves. To date, there is a paucity of data in the literature regarding the safety and efficacy of the EEA in the treatment of ECs of the ventral cranial base.METHODSThe authors reviewed a prospectively acquired database of EEAs for resection of ECs over 8 years at Weill Cornell, NewYork-Presbyterian Hospital. All procedures were performed by the senior authors. Standardized clinical and radiological parameters were assessed before and after surgery. Statistical tests were used to determine the impact of previous surgery and tumor volume on extent of resection and recurrence as well as the method of closure on rate of CSF leak.RESULTSBetween January 2009 and February 2017, 7 patients (4 males and 3 females; age range 16-70 years) underwent a total of 8 surgeries for EC resection utilizing the EEA. Transplanum and transclival extensions were performed in 3 and 5 patients, respectively. Methods of closure incorporated a gasket seal in 6 of 8 procedures and a nasoseptal flap in 7 of 8 procedures. Gross-total resection (GTR) was achieved in 43% of patients, and near-total resection (> 95%) was obtained in another 43%. Complications included diabetes insipidus (n = 2), postoperative CSF leak (n = 2), transient third cranial nerve palsy (n = 1), and epistaxis (n = 1). With a mean follow-up of 43.5 months, recurrence has been observed in 2 of 7 patients. In 1 case, reoperation for recurrence was required 71 months following the initial surgery. Use of the gasket-seal technique with nasoseptal flap coverage significantly correlated with the absence of postoperative CSF leakage (p = 0.018). GTR was achieved in 25% of the patients who had prior surgeries and in 50% of patients without previous resections. The mean volume of cysts in which GTR was achieved (4.3 ± 1.8 cm3) was smaller than that in which subtotal or near-total resection was achieved (12.2 ± 11 cm3, p = 0.134).CONCLUSIONSThe EEA for resection of ECs of the ventral cranial base is a safe and effective operative strategy that avoids crossing the plane of the cranial nerves. In the authors' experience, gasket-seal closure with nasoseptal flap coverage has been associated with a decreased risk of postoperative CSF leakage.

Quality of Life After Combined Endonasal Endoscopic Odontoidectomy and Posterior Suboccipital Decompression and Fusion.

BACKGROUND: Basilar invagination can result from systemic diseases that can weaken structural integrity of the craniocervical junction. Definitive treatment often requires ventral decompression and posterior decompression and fusion. Endonasal odontoidectomy is a relatively new minimal access procedure; quality of life (QOL) after this procedure has not been reported. METHODS: We reviewed a consecutive database of endonasal odontoidectomy cases and identified patients having posterior decompression and fusion. Two QOL questionnaires were administered postoperatively: Sino-Nasal Outcome Test and 36-Item Short Form Survey. Comparisons with other endonasal or Chiari procedures were performed. RESULTS: The study comprised 14 patients; 79% had Chiari malformation in addition to basilar invagination. Mean follow-up was 17.2 months. Symptomatic improvement occurred in 78.6% after surgery. Average postoperative Sino-Nasal Outcome Test scores were 39.2 ± 17.93, with worst scores in areas related to fatigue and sleep patterns but not nasal function. 36-Item Short Form Survey scores were lower in areas of physical function and general health but better for emotional health and pain. Compared with patients undergoing Chiari malformation surgery without endonasal odontoid resection or fusion, patients undergoing odontoidectomy had higher QOL in areas of role emotional, emotional well-being, and pain but worse QOL in general health and role physical. CONCLUSIONS: Patients undergoing posterior decompression and fusion with endonasal odontoidectomy do well after surgery with respect to nasal function and emotional health. Patients who also have severe basilar invagination associated with systemic diseases demonstrate reduced QOL after surgery in areas of physical function and sleep leading to fatigue, irritability, and concentration difficulty, likely related to their systemic disease.

Decision-making algorithm for minimally invasive approaches to anterior skull base meningiomas.

OBJECTIVE Anterior skull base meningiomas are benign lesions that cause neurological symptoms through mass effect on adjacent neurovascular structures. While traditional transcranial approaches have proven to be effective at removing these tumors, minimally invasive approaches that involve using an endoscope offer the possibility of reducing brain and nerve retraction, minimizing incision size, and speeding patient recovery; however, appropriate case selection and results in large series are lacking. METHODS The authors developed an algorithm for selecting a supraorbital keyhole minicraniotomy (SKM) for olfactory groove meningiomas or an expanded endoscopic endonasal approach (EEA) for tuberculum sella (TS) or planum sphenoidale (PS) meningiomas based on the presence or absence of olfaction and the anatomical extent of the tumor. Where neither approach is appropriate, a standard transcranial approach is utilized. The authors describe rates of gross-total resection (GTR), olfactory outcomes, and visual outcomes, as well as complications, for 7 subgroups of patients. Exceptions to the algorithm are also discussed. RESULTS The series of 57 patients harbored 57 anterior skull base meningiomas; the mean tumor volume was 14.7 ± 15.4 cm3 (range 2.2-66.1 cm3), and the mean follow-up duration was 42.2 ± 37.1 months (range 2-144 months). Of 19 patients with olfactory groove meningiomas, 10 had preserved olfaction and underwent SKM, and preservation of olfaction in was seen in 60%. Of 9 patients who presented without olfaction, 8 had cribriform plate invasion and underwent combined SKM and EEA (n = 3), bifrontal craniotomy (n = 3), or EEA (n = 2), and one patient without both olfaction and cribriform plate invasion underwent SKM. GTR was achieved in 94.7%. Of 38 TS/PS meningiomas, 36 of the lesions were treated according to the algorithm. Of these 36 meningiomas, 30 were treated by EEA and 6 by craniotomy. GTR was achieved in 97.2%, with no visual deterioration and one CSF leak that resolved by placement of a lumbar drain. Two patients with tumors that, based on the algorithm, were not amenable to an EEA underwent EEA nonetheless: one had GTR and the other had a residual tumor that was followed and removed via craniotomy 9 years later. CONCLUSIONS Utilizing a simple algorithm aimed at preserving olfaction and vision and based on maximizing use of minimally invasive approaches and selective use of transcranial approaches, the authors found that excellent outcomes can be achieved for anterior skull base meningiomas.

Residual enhancing disease after surgery for glioblastoma: evaluation of practice in the United Kingdom.

BACKGROUND: A growing body of clinical data highlights the prognostic importance of achieving gross total resection (GTR) in patients with glioblastoma. The aim of this study was to determine nationwide practice and attitudes towards achieving GTR and dealing with residual enhancing disease. METHODS: The study was in 2 parts: an electronic questionnaire sent to United Kingdom neuro-oncology surgeons to assess surgical practice followed by a 3-month prospective, multicenter observational study of current neurosurgical oncology practice. RESULTS: Twenty-seven surgeons representing 22 neurosurgical units completed the questionnaire. Prospective data were collected for 113 patients from 15 neurosurgical units. GTR was deemed to be achieved at time of surgery in 82% (91/111) of cases, but in only 45% (36/80) on postoperative MRI. Residual enhancing disease was deemed operable in 16.3% (13/80) of cases, however, no patient underwent early repeat surgery for residual enhancing disease. The most commonly cited reason (38.5%, 5/13) was perceived lack of clinical benefit. CONCLUSION: There is a subset of patients for whom GTR is thought possible, but not achieved at surgery. For these patients, early repeat resection may improve overall survival. Further prospective surgical research is required to better define the prognostic implications of GTR for residual enhancing disease and examine the potential benefit of this early re-intervention.

Corrigenda: Residual enhancing disease after surgery for glioblastoma: evaluation of practice in the United Kingdom.

[This corrects the article DOI: 10.1093/nop/npx023.][This corrects the article DOI: 10.1093/nop/npx023.].