Objective: The incidence of sporadic vestibular schwannoma (VS) has increased significantly over recent decades. The rising incidence of VS has been largely attributed to the increasing use of magnetic resonance imaging (MRI), especially with regard to incidentally diagnosed tumors. However, no study to date has directly investigated this supposed etiology beyond the observation that VS incidence rates have risen in the post-MRI era. Therefore, the primary objective of the current study was to characterize the incidence of head MRIs over the previous two decades in Olmsted County, Minnesota and compare this trend to the incidence of asymptomatic, incidentally diagnosed VS over the same time period. Study Design: Population-based incidence study. Setting/Patients: Using the unique resources of the Rochester Epidemiology Project, procedure codes for head MRIs and diagnostic codes for VS among residents of Olmsted County, Minnesota between Jan 1, 1995 and Dec 31, 2016 were retrieved. Incidence rates of head MRI and incidentally diagnosed VS were calculated on a per-year basis. Results: A total of 43,561 head MRIs among 30,002 distinct persons were identified from 1995 to 2016. The incidence of head MRI significantly increased between 1995 and 2003 (p < 0.001), but remained stable between 2004 and 2016 (p = 0.14). Over the same time interval, 25 cases of incidentally diagnosed VS were identified. The incidence of asymptomatic VS increased over time from 0.72 per 100,000 person-years between 1995 and 1999 to 1.29 between 2012 and 2016 (p = 0.058). No plateauing of incidence rates was observed in incidental tumors over the study period. The size of incidentally diagnosed tumors did not change over the study period (p = 0.93), suggesting that the increasing incidence of asymptomatic tumors is not explained by improved diagnostic capability of more recent MRI studies. Conclusions: Despite the plateauing of head MRI incidence rates after 2004, the incidence of asymptotic, incidentally diagnosed VS continued to increase. Our findings suggest that there may be additional contributory etiologies for the rising incidence of VS beyond greater detection alone.
Blood-Labyrinthine Barrier Permeability Assessed Using Postcontrast Delayed 3D-FLAIR MRI Imaging in Vestibular Schwannoma
Journal of Neurological Surgery Part B: Skull Base | February 5, 2020 | Volume: No volume available | Issue: No issue available | Pages: No page available
ObjectiveIgG4‐related disease (IgG4‐RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor‐like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4‐RD.MethodsReview of all cases at three tertiary‐referral centers since disease description in 2003.ResultsEleven patients were identified at a median age at presentation of 58 years (IQR, 38–65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4‐positive plasma cells per high‐powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy.ConclusionsIgG4‐RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.Level of Evidence4 Laryngoscope, 130:2574–2580, 2020
Dose Reduction for Sinus and Temporal Bone Imaging Using Photon-Counting Detector CT With an Additional Tin Filter
Objective The aim of this study was to quantitatively demonstrate radiation dose reduction for sinus and temporal bone examinations using high-resolution photon-counting detector (PCD) computed tomography (CT) with an additional tin (Sn) filter. Materials and Methods A multienergy CT phantom, an anthropomorphic head phantom, and a cadaver head were scanned on a research PCD-CT scanner using ultra-high-resolution mode at 100-kV tube potential with an additional tin filter (Sn-100 kV) and volume CT dose index of 10 mGy. They were also scanned on a commercial CT scanner with an energy-integrating detector (EID) following standard clinical protocols. Thirty patients referred to clinically indicated sinus examinations, and two patients referred to temporal bone examinations were scanned on the PCD-CT system after their clinical scans on an EID-CT. For the sinus cohort, PCD-CT scans were performed using Sn-100 kV at 4 dose levels at 10 mGy (n = 9), 8 mGy (n = 7), 7 mGy (n = 7), and 6 mGy (n = 7), and the clinical EID-CT was performed at 120 kV and 13.7 mGy (mean CT volume dose index). For the temporal bone scans, PCD-CT was performed using Sn-100 kV (10.1 mGy), and EID-CT was performed at 120 kV and routine clinical dose (52.6 and 66 mGy). For both PCD-CT and EID-CT, sinus images were reconstructed using H70 kernel at 0.75-mm slice thickness, and temporal bone images were reconstructed using a U70 kernel at 0.6-mm slice thickness. In addition, iterative reconstruction with a dedicated sharp kernel (V80) was used to obtain high-resolution PCD-CT images from a sinus patient scan to demonstrate improved anatomic delineation. Improvements in spatial resolution from the dedicated sharp kernel was quantified using modulation transfer function measured with a wire phantom. A neuroradiologist assessed the H70 sinus images for visualization of critical anatomical structures in low-dose PCD-CT images and routine-dose EID-CT images using a 5-point Likert scale (structural detection obscured and poor diagnostic confidence, score = 1; improved anatomic delineation and diagnostic confidence, score = 5). Image contrast and noise were measured in representative regions of interest and compared between PCD-CT and EID-CT, and the noise difference between the 2 acquisitions was used to estimate the dose reduction in the sinus and temporal bone patient cohorts. Results The multienergy phantom experiment showed a noise reduction of 26% in the Sn-100 kV PCD-CT image, corresponding to a total dose reduction of 56% compared with EID-CT (clinical dose) without compromising image contrast. The PCD-CT images from the head phantom and the cadaver scans demonstrated a dose reduction of 67% and 83%, for sinus and temporal bone examinations, respectively, compared with EID-CT. In the sinus cohort, PCD-CT demonstrated a mean dose reduction of 67%. The 10- and 8-mGy sinus patient images from PCD-CT were significantly superior to clinical EID-CT for visualization of critical sinus structures (median score = 5 ± 0.82 and P = 0.01 for lesser palatine foramina, median score = 4 ± 0.68 and P = 0.039 for nasomaxillary sutures, and median score = 4 ± 0.96 and P = 0.01 for anterior ethmoid artery canal). The 6- and 7-mGy sinus patient images did not show any significant difference between PCD-CT and EID-CT. In addition, V80 (sharp kernel, 10% modulation transfer function = 18.6 cm −1 ) PCD-CT images from a sinus patient scan increased the conspicuity of nasomaxillary sutures compared with the clinical EID-CT images. The temporal bone patient images demonstrated a dose reduction of up to 85% compared with clinical EID-CT images, whereas visualization of inner ear structures such as the incudomalleolar joint were similar between EID-CT and PCD-CT. Conclusions Phantom and cadaver studies demonstrated dose reduction using Sn-100 kV PCD-CT compared with current clinical EID-CT while maintaining the desired image contrast. Dose reduction was further validated in sinus and temporal bone patient studies. The ultra-high resolution capability from PCD-CT allowed improved anatomical delineation for sinus imaging compared with current clinical standard.
Assessing Nasal Soft-Tissue Envelope Thickness for Rhinoplasty
Importance Preoperative assessment of nasal soft-tissue envelope (STE) thickness is an important component of rhinoplasty that presently lacks validated tools. Objective To measure and assess the distribution of nasal STE thickness in a large patient population and to determine if facial plastic surgery clinicians can predict nasal STE thickness based on visual examination of the nose. Design, Setting, and Participants This retrospective review and prospective assessment of 190 adult patients by 4 expert raters was conducted at an academic tertiary referral center. The patients had high-resolution maxillofacial computed tomography (CT) scans and standardized facial photographs on file and did not have a history of nasal fracture, septal perforation, rhinoplasty, or other surgery or medical conditions altering nasal form. Data were analyzed in March 2019. Main Outcomes and Measures Measure nasal STE thickness at defined anatomic subsites using high-resolution CT scans. Measure expert-predicted nasal STE thickness based on visual examination of the nose using a scale from 0 (thinnest) to 100 (thickest). Results Of the 190 patients, 78 were women and the mean (SD) age was 45 (17) years. The nasal STE was thickest at the sellion (mean [SD]) (6.7 [1.7] mm), thinnest at the rhinion (2.1 [0.7] mm), thickened over the supratip (4.8 [1.0] mm) and nasal tip (3.1 [0.6] mm), and thinned over the columella (2.6 [0.4] mm). In the study population, nasal STE thickness followed a nearly normal distribution for each measured subsite, with the majority of patients in a medium thickness range. Comparison of predicted and actual nasal STE thickness showed that experts could accurately predict nasal STE thickness, with the highest accuracy at the nasal tip ( r , 0.73; prediction accuracy, 91%). A strong positive correlation was noted among the experts’ STE estimates ( r , 0.83-0.89), suggesting a high level of agreement between individual raters. Conclusions and Relevance There is variable thickness of the nasal STE, which influences the external nasal contour and rhinoplasty outcomes. With visual analysis of the nose, experts can agree on and predict nasal STE thickness, with the highest accuracy at the nasal tip. These data can aid in preoperative planning for rhinoplasty, allowing implementation of preoperative, intraoperative, and postoperative strategies to optimize the nasal STE, which may ultimately improve patient outcomes and satisfaction. Level of Evidence NA .
Involvement of the Cochlear Aqueduct by Jugular Paraganglioma Is Associated With Sensorineural Hearing Loss
Objective: The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. Study Design: Blinded, retrospective review of imaging and audiometry. Setting: Tertiary center. Patients: Adults with JP. Intervention(s): None. Main Outcome Measures: Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. Results: Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p < 0.0001). Regression analysis demonstrates that enhancement within the CA is associated with a BCPTA difference of 19.4 dB HL (p = 0.0006). Conclusions: Cochlear aqueduct involvement by JP is associated with SNHL in the absence of inner ear fistula, vestibulocochlear nerve involvement, or brainstem compression. Correlation with operative findings or histopathologic evidence of tumor involvement may validate this intriguing imaging finding.
Facial nerve venous malformation: A radiologic and histopathologic review of 11 cases
ObjectiveThe purpose of this article was to provide a combined pathologic and radiologic review of previous pathologically diagnosed facial nerve “hemangiomas” to confirm that these lesions are most characteristic of venous malformations rather than neoplasms.Study DesignRetrospective radiologic, clinical, and histopathologic review of all patients with a previous pathologically diagnosed facial nerve hemangioma of the temporal bone who underwent computed tomography or magnetic resonance imaging (MRI) were included. A consensus radiologic review for characteristic features and pathologic analysis was performed.Materials and MethodsA panel of 4 neuroradiologists retrospectively analyzed CT and MRI exams for 11 facial nerve hemangiomas and provided a consensus agreement on the characteristic imaging features. Concurrently, two neuropathologists reevaluated archived tissue specimens from these lesions and applied additional immunohistochemical and histochemical stains including D240, CD31, smooth muscle actin (SMA), Verhoeff Van Gieson (VVG) and glucose transporter 1 (GLUT1).ResultsLesions were composed of dilated vascular spaces with a simple, CD31‐positive endothelial lining and a smooth muscle component. All lesions were negative for markers found in arterial and lymphatic malformations and infantile hemangiomas. They had characteristic radiologic features previously ascribed to facial nerve hemangiomas. Namely, these lesions are typically T1 isointense or hypointense and T2 hyperintense relative to cerebral cortex and heterogeneously enhance on MRI. Bony canal expansion and erosion, intralesional calcification, and intracranial extension are common.ConclusionsOn the basis of this radiologic and pathologic review, these lesions are best characterized as venous malformations.Level of Evidence4
Expansile Traumatic Neuroma of the Intratemporal Facial Nerve
Objectives To present a rare case of traumatic facial neuroma involving the geniculate ganglion and review relevant literature. Patient Thirty-year-old man. Intervention Microsurgical resection via combined mastoid-middle fossa approach with great auricular nerve interpositional graft. Main Outcome Measures Patient demographics and pre- and postoperative facial nerve function. Results A 30-year-old man with a reported history of prior Bell's palsy developed progressive complete (House–Brackmann VI) right facial paralysis following blunt trauma. Imaging was strongly suggestive of a geniculate ganglion hemangioma. As the patient had no spontaneous improvement in his poor facial function over the course of 9 months, he underwent resection of the facial nerve lesion with great auricular nerve graft interposition via a combined mastoid-middle fossa approach. Histopathology demonstrated disorganized fascicles, with axonal clustering reminiscent of sprouting/regeneration following trauma. No cellular proliferation or vascular malformation was present. Conclusion Traumatic facial nerve neuromas can occur following temporal bone trauma and can closely mimic primary facial nerve tumors. Akin to the management of geniculate ganglion hemangioma and schwannoma, preoperative facial function largely dictates if and when surgery should be pursued.
Pattern of cochlear obliteration after vestibular Schwannoma resection according to surgical approach
Objectives/HypothesisTo investigate the prevalence and course of cochlear obliteration according to microsurgical approach to inform clinical decision making regarding optimal timing of cochlear implantation.Study DesignRetrospective radiologic review and chart review.MethodsPatients who underwent microsurgical resection of vestibular schwannoma (VS) with a minimum of two available postoperative magnetic resonance imaging (MRI) scans were analyzed. The prevalence and timing of cochlear and labyrinthine obliteration was classified using relevant MRI sequences.ResultsMRI studies in 60 patients were analyzed: 20 translabyrinthine (TL), 20 retrosigmoid, and 20 middle fossa (MF) cases. The first and last postoperative MRI was obtained a median of 3.4 months (interquartile range (IQR), 3.0–3.7 months) and 35 months (IQR, 27–83 months) after surgery, respectively. At the time of the first postoperative MRI, 21 (35%) patients had partial basal turn obliteration, and none of the patients had complete basal turn obliteration. At the time of the last postoperative MRI, six (10%) patients had partial basal turn obliteration and 17 (28%) patients had complete basal turn obliteration. The pattern of partial or complete basal turn obliteration differed significantly among all three surgical approaches (P < .001). Specifically, the risk of partial or complete obliteration of the basal turn was highest in the TL cohort and lowest in the MF cohort.ConclusionsThe prevalence and timing of cochlear obliteration after VS microsurgery varies significantly according to surgical approach. The risk of early and complete obliteration is highest in the TL group and lowest in the MF cohort. These data may inform clinical decision making regarding optimal timing of cochlear implantation in patients with advanced hearing loss after microsurgical resection.Level of Evidence4 Laryngoscope, 130:474–481, 2020
Skull Base Manifestations of Erdheim-Chester Disease: A Case Series and Systematic Review
Journal of Neurological Surgery Part B: Skull Base | March 6, 2019 | Volume: No volume available | Issue: No issue available | Pages: No page available