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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.

OBJECTIVE The supplemented Spetzler-Martin (Supp-SM) grading system was developed to improve the predictive accuracy of surgical risk for patients with brain arteriovenous malformations (AVMs). The aim of this study was to apply the Supp-SM grading system to patients having stereotactic radiosurgery (SRS) for Spetzler-Martin (SM) intermediate- (grade III) or high-grade (grade IV–V) AVMs to enable comparison with published microsurgical series. METHODS In 219 patients who underwent SRS during the period from 1990 to 2016, the Supp-SM grade was calculated for SM grade III (n = 154) or SM grade IV–V (n = 65) AVMs. The Supp-SM grades in these patients were 4 (n = 14, 6%), 5 (n = 36, 16%), 6 (n = 67, 31%), 7 (n = 76, 35%), and 8–9 (n = 26, 12%). Sixty patients (27%) had deep AVMs (basal ganglia, thalamus, or brainstem). Thirty-nine patients (18%) had volume-staged SRS; 41 patients (19%) underwent repeat SRS. The median follow-up period was 69 months for SM grade III AVMs and 113 months for SM grade IV–V AVMs. RESULTS AVM obliteration was confirmed in 163 patients (74%) at a median of 38 months after initial SRS. The obliteration rates at 4 and 8 years were 59% and 76%, respectively. Thirty-one patients (14%) had post-SRS deficits from hemorrhage (n = 7, 3%) or radiation injury (n = 24, 11%). Six patients (3%) died after SRS (hemorrhage, n = 5; radiation injury, n = 1). The rates of neurological decline or death at 4 and 8 years were 11% and 18%, respectively. Factors predictive of nonobliteration were deep location (HR 0.57, 95% CI 0.39–0.82, p = 0.003) and increasing AVM volume (HR 0.96, 95% CI 0.93–0.99, p = 0.002). Increasing AVM volume was the only factor associated with neurological decline (HR 1.05, 95% CI 1.02–1.08, p = 0.002). The Supp-SM grading score did not correlate with either obliteration (HR 0.94, 95% CI 0.82–1.09, p = 0.43) or neurological decline (HR 1.15, 95% CI 0.84–1.56, p = 0.38). CONCLUSIONS The Supp-SM grading system was not predictive of outcomes after SRS of intermediate- or high-grade AVM. In a cohort that included a high percentage (47%) of "inoperable" AVMs according to Supp-SM grade (≥ 7), most patients had obliteration after SRS, although there was a high risk of neurological decline.

The lingual process of the sphenoid bone (LP) and the petrolingual ligament (PLL) surround laterally the internal carotid artery within the middle cranial fossa (MCF).To study the LP and the PLL and anatomical variations considering their relationships with different structures and landmarks within the MCF, especially oriented toward the endoscopic endonasal approaches.Seventy-two sides of dry skulls and 20 sides of embalmed specimens were studied. The measurements of the LP and the PLL were obtained, considering important landmarks in the MCF.The LP had a mean length and height of 5 mm and 3 mm, respectively. Its distance from the foramen lacerum was 6 mm, from the foramen ovale 10 mm, foramen rotundum 15 mm, and petrous apex 9 mm. In 44 sides (61.11%), the LP partially closed the lateral aspect of the carotid sulcus; in 17 sides (23.61%), it was found as a near-ring; and in 11 sides (15.2%), it was considered rudimentary. Considering the PLL, its length and height were, respectively, 9 mm, and 4 mm.The LP and PLL separate the carotid artery at the inferior aspect of Meckel's cave and constitute important landmarks for endoscopic endonasal approaches to Meckel's cave and MCF, and their identification and removal is essential for internal carotid artery mobilization in this area.

Spontaneous tumor shrinkage during wait-and-scan management of sporadic vestibular schwannoma is generally considered an uncommon phenomenon. However, most data informing this understanding stem from single-slice linear tumor measurements taken in the axial imaging plane. The objective of the current work was to characterize the regression capacity of sporadic vestibular schwannomas using volumetric tumor measurements.Retrospective cohort study using slice-by-slice, three-dimensional volumetric tumor measurements.Three tertiary referral centers.Patients with sporadic vestibular schwannoma.Wait-and-scan.Regression-free survival rates with regression defined as a decrease of at least 20% of the tumor volume.Among 952 patients undergoing a total of 3,505 magnetic resonance imaging studies during observation, 123 experienced volumetric tumor regression after diagnosis at a median of 1.2 years (interquartile range, 0.6-2.9 yr). Volumetric regression-free survival rates (95% confidence interval; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (92-95%; 662), 86% (83-89%; 275), and 78% (73-82%; 132), respectively. Among 405 patients who demonstrated an initial period of tumor growth but continued wait-and-scan management, 48 experienced volumetric regression at a median of 1.2 years (interquartile range, 0.8-2.6 yr) after initial growth. Volumetric regression-free survival rates at 1, 3, and 5 years after initial growth were 94% (92-97%; 260), 84% (79-89%; 99), and 75% (67-83%; 43), respectively. Ultimately, only 82 of the 952 patients studied showed exclusively volumetric tumor regression (i.e., without any periods of tumor growth) by the time of last follow-up.Spontaneous volumetric tumor shrinkage during wait-and-scan management occurs more frequently than suggested by previous studies using linear tumor measurements and can even occur after previous episodes of documented tumor growth. These data further highlight the dynamic nature of vestibular schwannoma growth. To this end, the application of natural history data to patient management requires a nuanced approach that parallels the complex tumor behavior of vestibular schwannoma.

Develop a predictive model for incomplete microsurgical resection of sporadic vestibular schwannoma (VS).Historical cohort.Tertiary referral center.Patients with sporadic VS.Microsurgery with preoperative intent of gross total resection.Patient and tumor characteristics that influence extent of resection.Among 603 patients, 101 (17%) had intracanalicular tumors and 502 (83%) had tumors with cerebellopontine angle (CPA) extension. For patients with CPA tumors, 331 (66%) underwent gross total resection and 171 (34%) underwent near-total or subtotal resection (NTR-STR). Multivariable modeling identified older age at surgery, larger linear tumor size, and absence of a fundal fluid cap as predictive of NTR-STR ( p0.001). From this model, one can estimate that a 20-year-old with a tumor that has less than 10 mm of CPA extension and a present fundal fluid cap has a predicted probability of NTR-STR of 0.01 (or 1%), whereas a 70-year-old with a tumor that has 30 mm or greater CPA extension and absence of a fundal fluid cap has a predicted probability of NTR-STR of 0.91 (or 91%). Among the 171 patients who underwent NTR-STR, 24 required secondary treatment at the time of last follow-up.The primary predictors of incomplete microsurgical resection of VS include older age at surgery, larger linear tumor size, and absence of a fundal fluid cap. These factors can be used to estimate the likelihood of NTR-STR, aiding in preoperative discussions regarding future surveillance and potential need of secondary treatment, as well as shared clinical decision making.

OBJECTIVE Facial nerve palsy is a debilitating condition that can arise from iatrogenic, traumatic, or congenital causes. One treatment to improve function of the facial muscles after facial nerve injury is hypoglossal-to-facial nerve anastomosis (HFA). HFA’s efficacy and predictors of its success vary in the literature. Here, the authors present a patient-level analysis of a literature-based cohort to assess outcomes and investigate predictors of success for HFA. METHODS Seven electronic databases were queried for studies providing baseline characteristics and outcomes of patients who underwent HFA. Postoperative outcomes were measured using the House-Brackmann (HB) grading scale. A change in HB grade of 3 points or more was classified as favorable. A cutoff value for time to anastomosis associated with a favorable outcome was determined using the Youden Index. RESULTS Nineteen articles with 157 patients met the inclusion criteria. The mean follow-up length was 27.4 months, and the mean time to anastomosis after initial injury was 16 months. The end-to-side and end-to-end anastomosis techniques were performed on 84 and 48 patients, respectively. Of the 130 patients who had available preoperative and postoperative HB data, 60 (46.2%) had a favorable outcome. Time from initial injury to anastomosis was significantly different between patients with favorable and unfavorable outcomes (7.3 months vs 29.2 months, respectively; p < 0.001). The optimal cutoff for time to anastomosis to achieve a favorable outcome was 6.5 months (area under the curve 0.75). Patients who underwent anastomosis within 6.5 months of injury were more likely to achieve a favorable outcome (73% vs 31%, p < 0.001). CONCLUSIONS HFA is an effective method for restoring facial nerve function. Favorable outcomes for facial nerve palsy may be more likely to occur when time to anastomosis is within a 6.5-month window.

Chalazia are benign eyelid lesions caused by the obstruction and inflammatory reaction of the meibomian glands. Demodex mites are one potential cause of chalazia leading to mechanical obstruction of the meibomian gland. In this prospective randomized study, we examine a novel approach to treating chalazia with the use of microblepharoexfoliation (MBE), an in-office lid hygiene technique that exfoliates the eyelid margins.Fifty patients with clinical evidence of acute chalazion were enrolled in this study. Subjects were randomly assigned to a MBE plus lid hygiene group (23 patients, mean age 66.6 ± 16.6 years) or a lid hygiene alone group (27 patients, mean age 62.1 ± 14.4). The MBE plus lid hygiene group received MBE treatment and were evaluated 1 month after the baseline visit. The main outcome measured was the resolution of the chalazion at the 1-month follow-up visit.The lid hygiene plus MBE treatment group demonstrated a statistically significant resolution of the chalazion compared with the lid hygiene group alone ( P = 0.007; chi-square test). Among the MBE plus hygiene group, 87% of the patients had resolution of their chalazion as opposed to the lid hygiene alone group, which had 44% resolution.This is the first prospective, randomized clinical trial that demonstrated efficacy of MBE as a noninvasive adjunctive treatment method for chalazion resolution.

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. The goal of this study was to update our oncologic outcomes for this disease and explore prognostic factors associated with survival.We performed a retrospective analysis of patients with ENB treated at a single tertiary care institution from January 1, 1960, to January 1, 2020. Univariate and multivariate analysis was performed. Overall survival (OS), progression-free survival (PFS), and distant metastasis-free survival (DMFS) were reported.Among 143 included patients, the 5-year OS was 82.3% and the 5-year PFS was 51.6%; 5-year OS and PFS have improved in the modern era (2005-present). Delayed regional nodal metastasis was the most common site of recurrence in 22% of patients (median, 57 months). On univariate analysis, modified Kadish staging (mKadish) had a negative effect on OS, PFS, and DMFS (p 0.05). Higher Hyams grade had a negative effect on PFS and DMFS (p 0.05). Positive margin status had a negative effect on PFS (p 0.05). Orbital invasion demonstrated worsening OS (hazard ratio, 3.1; p 0.05). On multivariable analysis, high Hyams grade (3 or 4), high mKadish stage (C+D), and increasing age were independent negative prognostic factors for OS (p 0.05). High Hyams grade (3+4), high mKadish stage (C+D), age, and positive margin status were independent negative prognostic factors for PFS (p 0.05). High Hyams grade (3+4) was an independent negative prognostic factor for DMFS (p 0.05).Patients with low Hyams grade and mKadish stage have favorable 5-year OS, PFS, and DMFS.

Selection of skull base approaches is a critical skill for complex cranial surgery, which demands nuanced understanding of neuroanatomy and pathology.To develop novel pedagogical resources for approach selection education and assessment.A prospectively maintained skull base registry was screened for posterior fossa tumors amenable to 3-dimensional (3D) modeling of multiple operative approaches. Inclusion criteria were high-resolution preoperative and postoperative computed tomography and MRI studies (≤1 mm) and consensus that at least 3 posterior fossa craniotomies would provide feasible access. Cases were segmented using Mimics and modeled using 3-Matic. Clinical Vignettes, Approach Selection Questionnaire, and Clinical Application Questionnaire were compiled for implementation as a teaching/testing tool.Seven cases were selected, each representing a major posterior fossa approach group. 3D models were rendered using clinical imaging for the primary operative approach, as well as a combination of laboratory neuroanatomic data and extrapolation from comparable craniotomies to generate 2 alternative approaches in each patient. Modeling data for 3D figures were uploaded to an open-sourced database in a platform-neutral fashion (.x3d) for virtual/augmented reality and 3D printing applications. A semitransparent model of each approach without pathology and with key deep structures visualized was also modeled and included for comprehensive understanding.We report a novel series of open-source 3D models for skull base approach selection training, with supplemental resources. To the best of our knowledge, this is the first such series designed for pedagogical purposes in skull base surgery or centered on open-source principles.

OBJECTIVE There is a reported elevated risk of cerebral aneurysms in patients with intracranial dural arteriovenous fistulas (dAVFs). However, the natural history, rate of spontaneous regression, and ideal treatment regimen are not well characterized. In this study, the authors aimed to describe the characteristics of patients with dAVFs and intracranial aneurysms and propose a classification system. METHODS The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database from 12 centers was retrospectively reviewed. Analysis was performed to compare dAVF patients with (dAVF+ cohort) and without (dAVF-only cohort) concomitant aneurysm. Aneurysms were categorized based on location as a dAVF flow-related aneurysm (FRA) or a dAVF non–flow-related aneurysm (NFRA), with further classification as extra- or intradural. Patients with traumatic pseudoaneurysms or aneurysms with associated arteriovenous malformations were excluded from the analysis. Patient demographics, dAVF anatomical information, aneurysm information, and follow-up data were collected. RESULTS Of the 1077 patients, 1043 were eligible for inclusion, comprising 978 (93.8%) and 65 (6.2%) in the dAVF-only and dAVF+ cohorts, respectively. There were 96 aneurysms in the dAVF+ cohort; 10 patients (1%) harbored 12 FRAs, and 55 patients (5.3%) harbored 84 NFRAs. Dural AVF+ patients had higher rates of smoking (59.3% vs 35.2%, p < 0.001) and illicit drug use (5.8% vs 1.5%, p = 0.02). Sixteen dAVF+ patients (24.6%) presented with aneurysm rupture, which represented 16.7% of the total aneurysms. One patient (1.5%) had aneurysm rupture during follow-up. Patients with dAVF+ were more likely to have a dAVF located in nonconventional locations, less likely to have arterial supply to the dAVF from external carotid artery branches, and more likely to have supply from pial branches. Rates of cortical venous drainage and Borden type distributions were comparable between cohorts. A minority (12.5%) of aneurysms were FRAs. The majority of the aneurysms underwent treatment via either endovascular (36.5%) or microsurgical (15.6%) technique. A small proportion of aneurysms managed conservatively either with or without dAVF treatment spontaneously regressed (6.2%). CONCLUSIONS Patients with dAVF have a similar risk of harboring a concomitant intracranial aneurysm unrelated to the dAVF (5.3%) compared with the general population (approximately 2%–5%) and a rare risk (0.9%) of harboring an FRA. Only 50% of FRAs are intradural. Dural AVF+ patients have differences in dAVF angioarchitecture. A subset of dAVF+ patients harbor FRAs that may regress after dAVF treatment.

Background Jugular paragangliomas represent a surgical challenge due to their vascularity and proximity to vital neurovascular structures. Preoperative embolization aids in reducing intraoperative blood loss, transfusion requirements, and improves surgical visualization. Several embolization agents have been used. Objective The aim of this study is to evaluate the safety and efficacy of PVA in pre-operative embolization of jugular paragangliomas. Methods A retrospective review of all patients who underwent jugular paraganglioma resection with pre-operative embolization between 2000 and 2020 was performed. Pre-operative data including baseline patient and tumor characteristics were documented. Outcomes of preoperative embolization including extent of devascularization and post-embolization complications were recorded. Early and long-term postoperative outcomes were reported. Results Twenty-nine patients met study criteria with a median age of 38 years. Average tumor size was 3.4±1.8 cm. The most commonly encountered arterial feeder was the ascending pharyngeal artery followed by the posterior auricular artery. More than 50% reduction in tumor blush was achieved in 25 patients (86.2%). None of the patients experienced new or worsening cranial neuropathy following embolization. Gross total or Near total resection was achieved in 13 patients (44.8%). A STR or NTR was chosen in these patients to preserve cranial nerve function or large vessel integrity. Average intraoperative estimated blood loss was 888 ml, 9 patients (31%) required intra-operative transfusion of blood products. Extent of resection and post-operative complications did not correlate with extent of devascularization. Conclusion Pre-operative embolization of jugular paraganglioma tumors with PVA particles is an effective strategy with a high safety profile.