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1.
Otolaryngol Head Neck Surg ; 162(5): 666-673, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32178578

RESUMO

OBJECTIVE: Although granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is classically characterized by systemic disease involving the kidneys and airway, approximately 10% of patients who have it present with isolated central nervous system disease. When involving the skull base, GPA frequently mimics more common pathology, resulting in diagnostic challenges and delay. The primary objective of this study is to characterize the cranial base manifestations of GPA, highlighting aspects most relevant to the skull base surgeon. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic referral center. SUBJECTS AND METHODS: Retrospective analysis of all patients with skull base GPA treated at a tertiary referral center from January 1, 1996, to May 1, 2018. RESULTS: Twenty-nine patients met inclusion criteria. Twenty-one (72%) initially presented with skull base symptomatology as their cardinal manifestation of GPA. Twenty-four (82%) presented with cranial neuropathy at some point in their disease course. The trigeminal nerve was most commonly involved (12 of 24, 50%), followed by the facial (11 of 24, 46%) and optic (8 of 24, 33%) nerves. Eighteen patients reported hearing loss attributed to the GPA disease process, presenting as conductive, sensorineural, or mixed. The most common locations for GPA-derived inflammatory skull base disease on imaging included the cavernous sinus (12 of 29, 41%) and the orbit (7 of 29, 24%). CONCLUSION: Establishing the diagnosis of skull base GPA remains challenging. Cranial neuropathy is diverse in presentation and often mimics more common conditions. Imaging findings are also unpredictable and frequently nonspecific. Careful review of patient history, clinical presentation, serology and biopsy results, and imaging can reveal important clues toward the diagnosis.

2.
Otol Neurotol ; 41(6): 813-847, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32150020

RESUMO

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.

3.
Laryngoscope ; 130(2): 474-481, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30919457

RESUMO

OBJECTIVES/HYPOTHESIS: To investigate the prevalence and course of cochlear obliteration according to microsurgical approach to inform clinical decision making regarding optimal timing of cochlear implantation. STUDY DESIGN: Retrospective radiologic review and chart review. METHODS: Patients 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. RESULTS: MRI 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. CONCLUSIONS: The 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 EVIDENCE: 4 Laryngoscope, 130:474-481, 2020.

4.
Invest Radiol ; 55(2): 91-100, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31770297

RESUMO

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) 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.

5.
Clin Neuroradiol ; 2019 Dec 05.
Artigo em Inglês | MEDLINE | ID: mdl-31807810

RESUMO

Temporal bone microanatomy is a common source of consternation for radiologists. Serpentine foramina, branching cranial nerves, and bony canals containing often clinically relevant but often miniscule arterial branches may all cause confusion, even among radiologists familiar with temporal bone imaging. In some cases, the tiniest structures may be occult or poorly visualized, even on thin-slice computed tomography (CT) images. Consequently, such structures are often either ignored or mistaken for pathologic entities. Yet even the smallest temporal bone structures have significant anatomic and pathologic importance. This paper reviews the anatomy and function of the temporal bone aqueducts, canals, clefts, and nerves, as well as the relevant developmental, inflammatory, and neoplastic processes that affect each structure.

6.
Laryngoscope ; 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31841234

RESUMO

OBJECTIVE: IgG4-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. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven 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. CONCLUSIONS: IgG4-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 EVIDENCE: 4 Laryngoscope, 2019.

7.
JAMA Facial Plast Surg ; 21(6): 511-517, 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31486840

RESUMO

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.


Assuntos
Algoritmos , Nariz/anatomia & histologia , Exame Físico , Rinoplastia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Valores de Referência , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
8.
Otol Neurotol ; 40(9): 1230-1236, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31469795

RESUMO

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.

9.
Laryngoscope Investig Otolaryngol ; 4(3): 347-352, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31236470

RESUMO

Objective: The 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 Design: Retrospective 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 Methods: A 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). Results: Lesions 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. Conclusions: On the basis of this radiologic and pathologic review, these lesions are best characterized as venous malformations. Level of Evidence: 4.

10.
J Neurol Surg Rep ; 80(1): e10-e13, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30941279

RESUMO

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.

11.
Otol Neurotol ; 40(4): 517-528, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30870370

RESUMO

PURPOSE: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. METHODS: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. RESULTS: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk-75 yr)] were evaluated for recurrent facial nerve paralysis at the authors' institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2-20). The median nadir House-Brackmann score was 4, with a median recovery to House-Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1-420 d). Diagnostic evaluation confirmed Melkersson-Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. CONCLUSION: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.


Assuntos
Doenças do Nervo Facial/complicações , Nervo Facial/cirurgia , Paralisia Facial , Fossa Craniana Média/cirurgia , Descompressão Cirúrgica/métodos , Paralisia Facial/etiologia , Paralisia Facial/terapia , Herpes Zoster da Orelha Externa/complicações , Humanos , Síndrome de Melkersson-Rosenthal/complicações , Dissinergia Cerebelar Mioclônica/complicações , Recidiva Local de Neoplasia/cirurgia , Estudos Retrospectivos
12.
Neurosurgery ; 85(4): E693-E701, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-30828728

RESUMO

BACKGROUND: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses. OBJECTIVE: To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation. METHODS: Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors' institution since January 1, 1996 was also performed to supplement these data. RESULTS: Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%). CONCLUSION: Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and/or surgical debulking to relieve mass effect.


Assuntos
Doença de Erdheim-Chester/diagnóstico por imagem , Doença de Erdheim-Chester/terapia , Base do Crânio/diagnóstico por imagem , Corticosteroides/administração & dosagem , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Radiocirurgia/métodos , Radiocirurgia/tendências , Estudos Retrospectivos
13.
World Neurosurg ; 122: e285-e290, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30321684

RESUMO

OBJECTIVE: To present a case of spontaneous cerebrospinal fluid (CSF) otorrhea from a fallopian canal meningocele involving the geniculate fossa and review all cases of fallopian canal CSF leak reported in the literature with discussion of management and outcomes. METHODS: A 53-year-old woman with history of morbid obesity and hypertension presented to a tertiary care referral center with unilateral high-volume CSF otorrhea. High-resolution temporal bone computed tomography demonstrated significant dilatation of the geniculate fossa. Rates of postoperative facial paralysis and refractory CSF leak were reported for the present case and prior cases reported in the literature. RESULTS: Locations of fallopian canal dehiscence, surgical approaches, techniques for packing dehiscence, rates of postoperative facial paralysis and CSF leak, and revision procedures were reported for the present case and 14 cases in the literature. The present case involved dehiscence of the geniculate fossa that was approached via combined transmastoid-middle cranial fossa exploration with facial nerve monitoring. The area of dehiscence was carefully packed with temporalis fascia, muscle, and artificial dural substitute overlay to repair the CSF leak without injuring the facial nerve. Postoperatively, no facial weakness was noted; however, right-sided high-volume CSF otorrhea persisted. After discussing treatment options, the patient underwent subtotal petrosectomy and blind-sac closure of the external auditory canal the following day. This successfully resolved the CSF leak without causing facial nerve weakness. CONCLUSIONS: Fallopian canal meningocele is an exceedingly rare cause of CSF otorrhea. Successful repair requires precise packing of the dilated facial canal to occlude the leak without injuring the facial nerve. For refractory CSF leak, subtotal petrosectomy and closure of the external auditory canal warrants consideration.


Assuntos
Otorreia de Líquido Cefalorraquidiano/etiologia , Otorreia de Líquido Cefalorraquidiano/cirurgia , Meningocele/complicações , Meningocele/cirurgia , Otorreia de Líquido Cefalorraquidiano/diagnóstico por imagem , Nervo Facial , Feminino , Humanos , Meningocele/diagnóstico por imagem , Pessoa de Meia-Idade , Osso Temporal
14.
Otol Neurotol ; 39(9): e849-e855, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30199501

RESUMO

OBJECTIVES: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. STUDY DESIGN: Multicenter retrospective review. SETTING: Three tertiary referral centers. PATIENTS: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. INTERVENTION: Surgical repair or observation. MAIN OUTCOME MEASURE: Radiographic findings and clinical symptoms were analyzed. RESULTS: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. CONCLUSIONS: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.


Assuntos
Cavidades Cranianas/diagnóstico por imagem , Perda Auditiva/classificação , Doenças do Labirinto/classificação , Canais Semicirculares/diagnóstico por imagem , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Cavidades Cranianas/cirurgia , Feminino , Perda Auditiva/diagnóstico por imagem , Perda Auditiva/cirurgia , Humanos , Doenças do Labirinto/diagnóstico por imagem , Doenças do Labirinto/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Canais Semicirculares/cirurgia , Adulto Jovem
15.
Otol Neurotol ; 39(9): e872-e875, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30080763

RESUMO

OBJECTIVE: Evaluate prevalence and pattern of occipital pneumatization (OP). PATIENTS: Individuals with imaging evidence of OP on computed tomography (CT). INTERVENTION(S): High resolution CT scans of the temporal bone. MAIN OUTCOME MEASURE: The prevalence and pattern of OP on 1000 CT scans performed at a large academic healthcare system. RESULTS: OP had a prevalence of 11.8% (n = 118) while occipital condyle pneumatization occurred in only 0.3%. Occipital air cells were right-sided in 27.1% (n = 32), left-sided in 51.7% (n = 61), and bilateral in 21.2% (n = 25), and OP was contiguous with the occipitomastoid suture. In cases of unilateral OP, the contralateral jugular foramen demonstrated relative enlargement (p = 0.006), but a direct association could not be established. CONCLUSIONS: The occipital bone is an accessory site of skull base pneumatization in 11.8% of the population undergoing temporal bone CT, while air cell extension into the occipital condyle occurs more rarely. These occipital air cells have a left-sided predilection and are always in direct proximity to the occipitomastoid suture, suggesting developmental egress from the mastoid. OP is a normal variant and lacks features of craniocervical pneumatization, which is a rare disorder.


Assuntos
Doenças Ósseas/epidemiologia , Osso Occipital/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto , Idoso , Doenças Ósseas/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência
17.
Otol Neurotol ; 39(8): e712-e721, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30001283

RESUMO

OBJECTIVE: To date, less than 150 cases of middle ear adenomatous neuroendocrine tumors (MEANTs) have been reported in the English literature. The objective of this study was to provide a contemporary analysis of these rare lesions and develop a consensus-driven staging system. STUDY DESIGN: Multi-institutional retrospective histopathologic, radiologic, and clinical review. SETTING: Six tertiary referral centers. PATIENTS: Thirty-two patients with pathologically confirmed MEANT. INTERVENTION: Surgical resection, adjuvant therapy. MAIN OUTCOME MEASURES: Clinical manifestations, outcomes, staging system. RESULTS: Patients commonly presented with progressive conductive or mixed hearing loss (90%), aural fullness (50%), and tonal tinnitus (46%). Pulsatile tinnitus (16%), carcinoid syndrome (4%), and facial nerve paresis (4%) were less commonly observed. MEANTs frequently mimicked temporal bone paraganglioma (31%) and cholesteatoma (15%) at presentation. According to a novel T/N/M/S staging system (S = secretory tumor) proposed herein, there were 6 (19%) T1, 19 (59%) T2, and 7 (22%) T3 MEANTs. T3 tumors were significantly more likely to undergo subtotal or near total resection compared with lower staged tumors secondary to adherence to critical neurovascular structures such as the petrous internal carotid artery and facial nerve (p = 0.027). Patients with T3 MEANTs were more likely to experience multiple recurrences, require adjuvant therapy with a somatostatin analogue, or develop permanent facial nerve paresis compared with lower staged tumors. At last follow up, no patients with T1 MEANTs had developed recurrence, whereas 37% (7/19) of patients with T2 MEANT and 57% (4/7) of patients with T3 MEANT experienced either disease recurrence after gross total resection (GTR) or regrowth of known residual tumor requiring additional surgery at a median duration of 72 months (95% CI, 24-84). CONCLUSIONS: Patients with MEANTs can present with nonspecific symptomatology that overlaps with more commonly encountered middle ear lesions. MEANT exhibits a proclivity for recurrence according to T-stage and long-term clinical follow up is necessary, particularly for advanced stage tumors.


Assuntos
Adenoma/patologia , Neoplasias da Orelha/patologia , Orelha Média/patologia , Tumores Neuroendócrinos/patologia , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasia Residual/patologia , Estudos Retrospectivos , Adulto Jovem
18.
Otolaryngol Head Neck Surg ; 159(4): 643-649, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29734937

RESUMO

Objectives Primary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL. Study Design Case series and chart review. Setting Tertiary referral center. Subjects and Methods Review of 48 patients with PSBL treated between 1994 and 2016. Results The median age at diagnosis was 60 years. Diffuse large B-cell lymphoma comprised the most common subtype (56%). Presenting symptoms included diplopia (52%), trigeminal hypesthesia (38%), headache (29%), facial nerve weakness (25%), B-symptoms (fevers, night sweats, and/or weight loss) (25%), and hearing loss (21%). Discrete lesions commonly mimicked meningioma, schwannoma, and nasopharyngeal carcinoma. Diffuse lesions imitated neurosarcoidosis, granulomatosis with polyangiitis, and carcinomatosis meningitis. Only 26% of all initial clinical evaluations suspected lymphoma. The combination of restricted diffusion on diffusion-weighted imaging, an absence of flow voids or surrounding hyperostosis, bony erosion and/or marrow signal replacement, and heterogeneous contrast enhancement facilitated delineation between PSBL and common skull base pathology. Cerebrospinal fluid (CSF) analysis was diagnostic of lymphoma in 24% of cases. Lymphoma within CSF portended significantly worse overall survival (85% vs 18% at 3-years; P < .001). Conclusion A history significant for multiple cranial nerve palsies, B-symptoms, and imaging findings inconsistent with common skull base pathology should raise suspicion for PSBL. CSF analysis in the setting of PSBL can provide diagnostic and prognostic value for patients.


Assuntos
Linfoma/patologia , Linfoma/terapia , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/terapia , Adulto , Idoso , Biópsia por Agulha , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Doenças do Sistema Nervoso Central/patologia , Doenças do Sistema Nervoso Central/terapia , Quimiorradioterapia/métodos , Estudos de Coortes , Terapia Combinada , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Estimativa de Kaplan-Meier , Linfoma/diagnóstico por imagem , Linfoma/mortalidade , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Imagem por Ressonância Magnética/métodos , Masculino , Meningioma/diagnóstico por imagem , Meningioma/patologia , Meningioma/terapia , Pessoa de Meia-Idade , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/terapia , Doenças Raras , Estudos Retrospectivos , Sarcoidose/diagnóstico por imagem , Sarcoidose/patologia , Sarcoidose/terapia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/mortalidade , Estatísticas não Paramétricas , Análise de Sobrevida
19.
Otolaryngol Head Neck Surg ; 159(3): 535-542, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29685084

RESUMO

Objective (1) Assess 3-dimensional volumetric growth of untreated sporadic vestibular schwannomas (VSs) in a large cohort of patients treated with conservative observation. (2) Compare volumetric and conventional linear diameter measurements for detecting tumor growth. Study Design Case series with chart review. Setting Tertiary skull base referral center. Subjects and Methods Patients with sporadic VS who elected initial conservative treatment with at least 2 serial magnetic resonance imaging (MRI) scans were included. Tumor volume was determined with 3-dimensional segmentation of MRI sequences. The volumetric threshold for tumor growth was an increase ≥20% from baseline tumor volume. Tumor size based on linear diameter was assessed with the 1995 American Academy of Otolaryngology-Head and Neck Surgery Foundation guidelines for VS outcome reporting, with growth defined as an increase ≥2 mm. Results A total of 361 patients were included with a median radiologic follow-up of 4.1 years (interquartile range [IQR], 2.5-6.8). At diagnosis, 232 VSs (64%) were purely intracanalicular, and 129 (36%) extended into the cerebellopontine angle. The median baseline tumor volume was 0.161 cm3 (IQR, 0.054-0.418). Overall, 69% of tumors demonstrated volumetric growth at a median of 1.1 years (IQR, 0.6-2.1) after initial MRI. In contrast, based on linear measurement assessment, 48% of tumors demonstrated growth at a median of 1.8 years (IQR, 0.8-3.1) from first MRI scan. Disequilibrium, facial hypoesthesia, aural fullness, initial tumor size, and nonincidental diagnosis were associated with tumor growth. Conclusion Three-dimensional volumetric assessment of VS provides a more sensitive measure of tumor growth when compared with linear diameter assessment. Through volumetric analysis, the current study revealed that a significant proportion of VSs demonstrate growth during observation.


Assuntos
Imageamento Tridimensional , Imagem por Ressonância Magnética/métodos , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/fisiopatologia , Carga Tumoral/fisiologia , Conduta Expectante/métodos , Centros Médicos Acadêmicos , Adulto , Idoso , Estudos de Coortes , Bases de Dados Factuais , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Neuroma Acústico/mortalidade , Neuroma Acústico/terapia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento
20.
Otol Neurotol ; 39(3): 372-377, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29342038

RESUMO

BACKGROUND: Gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) (T1WI) is the conventional imaging technique of choice to detect vestibular schwannoma (VS) recurrence or regrowth, despite suboptimal specificity secondary to enhancing postoperative changes. Furthermore, recent concerns regarding the accumulation of gadolinium in body tissues have led for a call to reduce the number of contrast-enhanced examinations. The objective of the current study is to evaluate the diagnostic accuracy of high-resolution noncontrast (three-dimensional [3D] T2) MRI relative to gadolinium-enhanced T1WI in the detection of VS recurrence after resection. METHODS: Following Institutional Review Board approval, 13 consecutive postoperative patients with VS recurrence or regrowth were identified from a prospectively maintained clinical database in which recurrence was determined by progression on serial postoperative MRI examination. Three blinded neuroradiologists retrospectively evaluated a total of 41 postoperative MRI examinations from these patients using only gadolinium-enhanced T1WI and 3D T2 MRI for recurrence or regrowth. Interobserver agreement, differences in detection between the two sequences, and the sensitivity and specificity of 3D T2 MRI were assessed. RESULTS: Fifteen of the 41 postoperative MRIs demonstrated progression, as determined by examiner consensus on the gadolinium-enhanced T1WI. Agreement, measured using Krippendorff's alpha, was 0.82 for the 3D T2 images and 0.83 for the contrast-enhanced T1WI. All the three examiners demonstrated no difference in the detection of progression between the two sequences (McNemar's test p values 0.69 for examiner 1, 0.63 for examiner 2, and 0.99 for examiner 3). The sensitivity of 3D T2 was 0.78 (CI 0.60-0.96), while the specificity was 0.94 (CI 0.86-1.00). CONCLUSION: Noncontrast high-resolution 3D T2 MRI seems sufficient to assess for recurrence or regrowth after VS resection. The results of this study have implications for reducing cost, time, and adverse events associated with gadolinium administration in this population requiring serial follow-up examinations. These promising, yet preliminary findings warrant confirmation with a larger prospective cohort of patients.


Assuntos
Imagem por Ressonância Magnética/métodos , Recidiva Local de Neoplasia/diagnóstico por imagem , Neuroma Acústico/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imageamento Tridimensional/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Adulto Jovem
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