Tratamiento multimodal en los schwanomas vestibulares grandes / Multimodal Treatment for Large Vestibular Schwannomas

Introducción: Los schwanomas vestibulares son lesiones clasificadas como grado I por la Organización Mundial de la Salud. Las opciones conductuales incluyen la observación, la radiocirugía o la resección microquirúrgica endoscópica. Objetivo: Describir el rol del tratamiento multimodal en los schwanomas vestibulares. Métodos: Se realizó una búsqueda sistemática de literatura médica para la identificación e inclusión de artículos en las siguientes bases de datos: PubMed/Medline, SciELO y Google Scholar, además de los servidores de preprints BioRvix y MedRvix. Se usaron los siguientes descriptores: vestibular schwanoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Se excluyeron editoriales, cartas al editor, libros, revisiones, metaanálisis y aquellos artículos con método deficiente de más de 20 años de publicación o sin mención al tema de interés. Fueron incluidos 16 artículos. Desarrollo: Los artículos incluyeron un total de 699 pacientes, de los cuales solo 228 (32,6 porciento) recibieron radioterapia en el período posoperatorio. El período de seguimiento promedió 49,6 meses. La preservación de la función facial se logró en el 88,3 porciento de los casos y el control tumoral en el 80,7 porciento. El estado de la preservación auditiva solo fue informado en el 50 porciento de los estudios. El uso de la radioterapia adyuvante mostró gran variabilidad. Conclusiones: El tratamiento multimodal para los schwanomas vestibulares grandes -compuesto por la resección subtotal, vaciamiento intratumoral, seguido de radiocirugía- se ha convertido en una opción plausible. Se necesita la publicación de mayor cantidad de reportes para ofrecer recomendaciones y estratificar la conducta(AU)

Introduction: Vestibular schwannomas are lesions classified as grade I by the World Health Organization. Behavioral options include observation, radiosurgery or endoscopic microsurgical resection. Objective: To describe the role of multimodal treatment for vestibular schwannomas. Methods: A systematic search of medical literature, for the identification and inclusion of articles, was carried out in the databases PubMed/Medline, SciELO and Google Scholar, as well as in the preprint servers BioRvix and MedRvix. The following descriptors were used: vestibular schwannoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Editorials, letters to the editor, books, reviews and metaanalyses were excluded, as well as articles with deficient method of more than 20 years of publication or not mentioning the topic of interest. Sixteen articles were included. Development: The articles included a total of 699 patients, of which only 228 (32.6 ) received radiotherapy in the postoperative period. The average follow-up period was 49.6 months. Preservation of facial function was achieved in 88.3 percent of cases; and tumor control, in 80.7 percent. Hearing preservation status was reported in only 50 percent of the studies. The usage of adjuvant radiotherapy showed great variability. Conclusions: Multimodal treatment for large vestibular schwannomas -composed of subtotal resection and intratumoral draining, followed by radiosurgery- has become a plausible option. The publication of a greater amount of reports is necessary to provide recommendations and stratify the behavior(AU)

Electroencephalographic microstates in vestibular schwannoma patients with tinnitus / 南方医科大学学报

OBJECTIVE@#To explore the biomarkers of tinnitus in vestibular schwannoma patients using electroencephalographic (EEG) microstate technology.@*METHODS@#The EEG and clinical data of 41 patients with vestibular schwannoma were collected. All the patients were evaluated by SAS, SDS, THI and VAS scales. The EEG acquisition time was 10-15 min, and the EEG data were preprocessed and analyzed using MATLAB and EEGLAB software package.@*RESULTS@#Of the 41 patients with vestibular schwannoma, 29 patients had tinnitus and 12 did not have tinnitus, and their clinical parameters were comparable. The average global explanation variances of the non-tinnitus and tinnitus groups were 78.8% and 80.1%, respectively. The results of EEG microstate analysis showed that compared with those without tinnitus, the patients with tinnitus had an increased frequency (P=0.033) and contribution (P=0.028) of microstate C. Correlation analysis showed that THI scale scores of the patients were negatively correlated with the duration of microstate A (R=-0.435, P=0.018) and positively with the frequencies of microstate B (R=0.456, P=0.013) and microstate C (R=0.412, P=0.026). Syntax analysis showed that the probability of transition from microstate C to microstate B increased significantly in vestibular schwannoma patients with tinnitus (P=0.031).@*CONCLUSION@#EEG microstate features differ significantly between vestibular schwannoma patients with and without tinnitus. This abnormality in patients with tinnitus may reflect the potential abnormality in the allocation of neural resources and the transition of brain functional activity.

Brazilian Society of Otology task force - Vestibular Schwannoma - evaluation and treatment

Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Hipoacusia sensorineural asimétrica en el diagnóstico de schwannoma vestibular / Asymmetric sensorineural hearing loss in the diagnosis of vestibular schwannoma

Introducción: se ha descrito que los pacientes con hipoacusia asimétrica cursan en un 2% con lesiones retrococleares. La conducta de escrutinio se ha basado en la audiometría. Existen varias definiciones de asimetría audiométrica descritas en la literatura, pero sin llegar a un consenso. Aunque la prueba de oro para el diagnóstico de schwannoma vestibular es la resonancia magnética con gadolíneo, la sospecha clínica se hace en base a la asimetría audiométrica. Objetivo: hacemos una revisión de los trabajos publicados al respecto en la literatu-ra y comentamos nuestra experiencia. Reflexión: queremos enfatizar en la importancia de estudiar a los pacientes con hipoacusia asimétrica con el fin de descartar patología retrococlear. Conclusión: a pesar de que no existe un consenso claro en la definición de hi-poacusia asimétrica, la sospecha clínica de un schwannoma vestibular se basa en la audiometría

Background: It is described that 2% of patients with asymmetric hearing loss have retrocochlear lesions. The scrutiny behavior has been based on audiometry. There are several definitions of audiometric asymmetry described in the literature, but without reaching a consensus. Although the gold standard for the diagnosis of vestibular schwannoma is gadolinium magnetic resonance imaging, the clinical sus-picion is based on audiometric asymmetry. Objective: we review the results published in this regard in the literature and com-ment on our own experience. Reflection: we want to emphasize the importance of studying asymmetric hearing loss patients in order to rule out retrocochlear etiology. Conclusions: although there is no clear consensus on the definition of asym-metric hearing loss, the clinical suspicion of a vestibular schwannoma is based on the audiometry.

Primera Radiocirugía de schwannoma vestibular en el sistema público uruguayo, Hospital Regional de Tacuarembó. Centro Oncológico del Norte

Resumen: Los schwannomas vestibulares son tumores benignos de lento crecimiento. Aproximadamente el 90% son de presentación unilateral, el 10% restante, son bilaterales y se encuentran asociados al síndrome de Neurofibromatosis tipo 2. Las opciones de tratamiento son observación, cirugía o radiocirugía. La radiocirugía es un tratamiento seguro, con altas tasas de control local y baja morbilidad en schwannomas vestibulares menores a 3 cm de diámetro mayor. Reportamos la primera radiocirugía realizada en el sistema público uruguayo para este tipo de patologías.

Abstract: Vestibular schwannomas are slow-growing benign tumors. Approximately 90% are unilateral, the remaining 10% are bilateral and are associated with Neurofibromatosis type 2 syndrome. Treatment options are observation, surgery or radiosurgery. Radiosurgery is a safe treatment, with high local control rates and low morbidity in vestibular schwannomas smaller than 3 cm in major diameter. We report the first radiosurgery performed in the Uruguayan public system for this type of pathology.

Resumo: Os schwannomas vestibulares são tumores benignos de crescimento lento. Aproximadamente 90% são unilaterais, os restantes 10% são bilaterais e estão associados à síndrome de neurofibromatose tipo 2. As opções de tratamento são observação, cirurgia ou radiocirurgia. A radiocirurgia é um tratamento seguro, com altas taxas de controle local e baixa morbilidade em schwannomas vestibulares com menos de 3 cm de diâmetro maior. Relatamos a primeira radiocirurgia realizada no sistema público uruguaio pra este tipo de patologia.

Resección microquirúrgica de schwannoma vestibular grado IVa con preservación de la función facial / Microsurgical resection of vestibular schwannoma grade IVa with preservation of facial function

Introducción: La cirugía de los schwannoma vestibulares constituye un desafío para los neurocirujanos. Debido a que se trata de un tumor benigno la resección completa de la lesión implica la curación del paciente. Sin embargo, este objetivo no siempre es fácil de lograr preservando la función de los nervios facial y acústico, especialmente en tumores de gran tamaño. Objetivos: Presentar detalles técnicos de la cirugía de resección de un schwannoma vestibular de gran tamaño (IVa) en el que se pudo preservar la función facial. Materiales y métodos: Se presenta el caso de una paciente femenina de 36 años que consultó por hipoacusia izquierda. En la RM preoperatoria se evidenciaba una lesión ocupante de espacio del ángulo pontocerebeloso izquierdo compatible con schwannoma vestibular con compresión del tronco encefálico y sin efecto de masa sobre el IV ventrículo (grado IVa). Mediante un abordaje suboccipital retromastoideo en posición de decúbito lateral se realizó la resección de la lesión en forma completa asistida por monitoreo del nervio facial. En todo momento se pudo preservar el plano aracnoideo que separaba el tumor de los nervios adyacentes. Resultados: Se logró una resección macroscópicamente completa con preservación de la función del nervio facial. La paciente permaneció internada por 96 hs en el postoperatorio sin complicaciones derivadas del procedimiento. Conclusión: La preservación del plano aracnoideo es un detalle técnico de mucha importancia para disminuir las posibilidades de lesión de los nervios facial y auditivo en la cirugía de resección de los schwannoma vestibulares.

Introduction: The surgery of vestibular schwannomas is a challenge for neurosurgeons.Because it is a benign tumor, complete resection of the lesion involves healing the patient. However, this objective is not always easy to achieve, preserving the function of both the facial and acoustic nerves, especially when dealing with large tumors. Objective: The objective of the video is to present some technical details of a large vestibular schwannoma (IVa) surgery in which the facial function could be preserved. Materials and methods: We present the case of a 36-year-old female patient who consulted for left hearing loss. The preoperative MRI showed a space- occupying lesion of the left pontocerebellar angle, which was compatible with vestibular schwannoma, with compression of the brainstem but with no mass effect on the IV ventricle (grade IVa). By means of a retromastoid suboccipital approach in the lateral prone position, the lesion was completely resected assisted by neurophysiological monitoring of the facial nerve. At all times, the arachnoid plane separating the tumor from the adjacent nerves was preserved. Results: A macroscopically complete resection was achieved preserving the facial nerve function. The patient stayed hospitalized for 96 hours during the postoperative period without any complication from the procedure. Conclusion: Preserving the arachnoid plane is a very important technical detail to reduce the possibilities of injury of the facial and auditory nerves in the vestibular schwannoma resection surgery.

Abordaje translaberíntico y rehabilitación vestibular: eficacia en el tratamiento de la disfunción vestibular en pacientes con schwannoma vestibular / Translabyrinthine approach and vestibular rehabilitation: efficacy in the treatment of vestibular dysfunction in patients with vestibular schwannoma

Introducción: En los schwannomas vestibulares (SV), la incidencia de disfunción vestibular como principal síntoma es del 10%, sin embargo, producen un notorio impacto en la calidad de vida de los pacientes. Objetivo: Evaluar el impacto funcional que produce la exéresis de SV por via translaberintica y la rehabilitación vestibular sobre los síntomas vestibulares, en los pacientes tratados en nuestro hospital. Material y Métodos: Se realizó un análisis prospectivo de 2 pacientes con diagnóstico de SV y disfunción vestibular agregada. En ambos pacientes, se realizó la resección a través de un abordaje translaberíntico. Los pacientes fueron evaluados funcionalmente antes y después de la cirugía. El impacto de la misma sobre la calidad de vida se evalúo mediante la Escala de Discapacidad por Mareo (DHI). Todos los pacientes realizaron un plan de rehabilitación vestibular personalizado y a los 12 meses postoperatorios presentaron una mejoría clínica significativa en comparación con el preoperatorio. Discusión: Las posibles opciones de tratamiento en estos pacientes resultan limitadas. En la literatura, se ha demostrado que la laberintectomía es un tratamiento eficaz para el tratamiento de síntomas vestibulares persistentes e incapacitantes, en los pacientes sin audición funcional. La calidad de vida de los mismos es reducida, y mejoraría significativamente después de la cirugía translaberintica y de un programa personalizado de rehabilitación vestibular. Conclusión: Debido a su eficacia comprobada, el abordaje translaberíntico y la resección de la lesión, acompañado de la realización un programa de rehabilitación vestibular, como estrategia de tratamiento para estos pacientes debe ser considerado.

Introduction: The incidence of disabling vestibular symptoms in vestibular schwannoma patients has been reported at roughly 10%. Despite their relative rarity, however, such symptoms can lead to physical and social limitations and reduce patients' quality of life. Unfortunately, published evidence on possible treatment options for vestibular schwannoma patients with disabling vestibular symptoms is limited. Objective: To report the functional impact of trans-labyrinthine microsurgery and vestibular rehabilitation, performed at our hospital, in two vestibular schwannoma patients with disabling vestibular symptoms. Methods: A prospective analysis was performed of two patients with a unilateral vestibular schwannoma, without serviceable hearing in their affected ear, and severely handicapped by attacks of rotatory vertigo and constant dizziness. Trans-labyrinthine surgery, with complete tumor resection, was performed in both patients. Preoperative and postoperative quality of life was measured using Dizziness Handicap Inventory (DHI) scores. Both patients also underwent a customized vestibular rehabilitation program and had a final evaluation 12 months post-operatively. Results: Relative to their preoperative evaluation, both patients experienced significant clinical improvement that persisted through 12 months of post-operative follow-up. Conclusions: Consistent with the few prior reports already published, we found that surgical removal of vestibular schwannomas by trans-labyrinthine surgery and vestibular rehabilitation was safe and effective for persistent, disabling vestibular symptoms. Postoperative vertigo and quality of life both improved significantly when microsurgery was combined with a personalized vestibular rehabilitation program. Disabling vestibular symptoms that impair quality of life in patients with vestibular schwannoma without serviceable hearing in the affected ear should be considered for trans-labyrinthine microsurgery and personalized vestibular rehabilitation.

Fresado de la fosa subarcuata para liberar la arteria cerebelosa anteroinferior en una cirugía de un schwannoma vestibular / Reaming the subarcuate fossa to release the anteroinferior cerebellar artery in surgery for a vestibular schwannoma

Introducción: El abordaje suboccipital retrosigmoideo es la vía principal para la resección de los schwannomas vestibulares (SV). La relación vascular más constante de los nervios del conducto auditivo interno es la arteria cerebelosa anteroinferior (ACAI); pudiendo su recorrido presentarse como un serio obstáculo para la resección completa de la lesión. Descripción del caso: Paciente varón de 38 años, con diagnóstico presuntivo de SV por resonancia magnética (Grado T3B). Se realiza cirugía por vía retrosigmoidea. Se observa a la ACAI totalmente recubierta por tejido dural y óseo, cuya liberación fue posible mediante fresado en la fosa subarcuata. Se logra una exéresis completa del tumor. El paciente evolucionó durante el estado posoperatorio sin déficit neurológico agregado. Discusión: La ACAI ha sido descripta fijada a la duramadre y/o incrustada en el hueso de la fosa subarcuata en escasas publicaciones, impidiendo la resección completa de un SV, especialmente de la porción intracanalicular. Sin embargo, su liberación supone riesgo adicional de lesión vascular. Conclusión: La lesión de la ACAI puede ser causal de alta morbilidad, por lo que el neurocirujano debe estar preparado para reconocer y resolver este tipo de situaciones.

Introduction: A retrosigmoid suboccipital approach is the route most commonly utilized to resect vestibular schwannomas (VS). However, the anterior inferior cerebellar artery (AICA) usually runs adjacent to internal auditory canal nerves, and its course may severely impede total tumor resection. Case report: A 38-year-old male patient presented with presumed grade T3B VS, diagnosed by magnetic resonance imaging (MRI). Surgery was performed using a retrosigmoid approach, during which the AICA was identified to be completely covered by dural and bone tissue. Further drilling in the subarcuate fossa was necessary to release the AICI, allowing for total gross resection of the VS. No neurological deficits were observed post-operatively. Discussion: On rare occasion, the AICA has been described fixed to the dura and/or embedded within subarcuate fossal bone, thereby preventing removal of the intra-canalicular portion of the VS and, hence, total resection. However, AICA release adds the risk of vascular injury. Conclusion: Injury to the AICA may cause high morbidity in patients with a vestibular schwannoma. Neurosurgeons must be able to recognize and deal with certain anatomical configurations that place patients at particularly-high risk.

Intratumoral Bleeding in a Vestibular Schwannoma

Vestibular schwannomas (VSs) account for 70% of all tumors of the cerebellopontine angle (CPA). Their clinical presentation is often insidious, with progressive hearing loss and involvement of other cranial nerves. Spontaneous hemorrhage in those tumors is very unusual, and generally presents with acute clinical features such as nausea, vomiting, headache and altered consciousness, usually with marked dysfunction of the cranial nerve involved, and with new deficits of neighboring cranial nerves. Asymptomatic patients are extremely rare. We present a case report of an incidental VS with asymptomatic bleeding, which evolved to death after surgery.

Schwannomas vestibulares (SVs) são responsáveis por cerca de 70% de todos os tumores do ângulo pontocerebelar. Sua apresentação costuma ser insidiosa, com perda auditiva progressiva e envolvimento de outros nervos cranianos. Hemorragia espontânea nesses tumores é incomum, e geralmente apresenta-se agudamente, com náusea, vômitos, cefaleia e alterações de consciência, normalmente com disfunção importante dos nervos cranianos envolvidos e com novos déficits dos nervos próximos. Pacientes assintomáticos são extremamente raros. Apresentamos um relato de caso de um SV incidental com sangramento assintomático que evoluiu para o óbito após cirurgia.

Peripheral Facial Palsy and Communicating Hydrocephalus as a Clinical Presentation of Hemorrhagic Vestibular Schwannoma: Case Report

The vestibular schwannoma is the most common extra-axial tumor of the posterior fossa. Hemorrhage associated with vestibular schwannomas has been described in less than 1% of the cases. The etiology remains unknown; however, some risk factors have been suggested, such as tumor size and tumor growth rate, the vascularity and histopathology of the tumor or even previous treatment with radiosurgery. The present case is of a 74-year-old female patient, who presented to our clinic in December of 2015 after a mild traumatic brain injury. In that context, she did a brain computed tomography (CT) scan and was diagnosed with a vestibular schwannoma­ an incidentaloma. It was decided at that time to treat the patient conservatively because of her comorbidities and the presentation of the disease. In March of 2017, the patient presented again to our clinic with a right peripheral facial paralysis (House-Brackmann [HB] grade IV-VI) and confusion. The CT scan revealed that bleeding around the vestibular schwannoma had caused the clinical presentation. We decided to treat the hydrocephalus with a ventriculoperitoneal shunt. At the time of her last follow-up visit, the confusion symptoms had resolved, and her right-sided facial function had improved to a HB grade II-VI.

Maximum diameter versus volumetric assessment for the response evaluation of vestibular schwannomas receiving stereotactic radiotherapy

PURPOSE: To explore the feasibility of maximum diameter as a response assessment method for vestibular schwannomas (VS) after stereotactic radiosurgery or fractionated stereotactic radiotherapy (RT), we analyzed the concordance of RT responses between maximum diameters and volumetric measurements. MATERIALS AND METHODS: Forty-two patients receiving curative stereotactic radiosurgery or fractionated stereotactic RT for VS were analyzed retrospectively. Twelve patients were excluded: 4 did not receive follow-up magnetic resonance imaging (MRI) scans and 8 had initial MRI scans with a slice thickness >3 mm. The maximum diameter, tumor volume (TV), and enhanced tumor volume (ETV) were measured in each MRI study. The percent change after RT was evaluated according to the measurement methods and their concordances were calculated with the Pearson correlation. The response classifications were determined by the assessment modalities, and their agreement was analyzed with Cohen kappa statistics. RESULTS: Median follow-up was 31.0 months (range, 3.5 to 86.5 months), and 90 follow-up MRI studies were analyzed. The percent change of maximum diameter correlated strongly with TV and ETV (r(p) = 0.85, 0.63, p = 0.000, respectively). Concordance of responses between the Response Evaluation Criteria in Solid Tumors (RECIST) using the maximum diameters and either TV or ETV were moderate (kappa = 0.58; 95% confidence interval, 0.32-0.85) or fair (kappa = 0.32; 95% confidence interval, 0.05-0.59), respectively. CONCLUSION: The percent changes in maximum diameter and the responses in RECIST were significantly concordant with those in the volumetric measurements. Therefore, the maximum diameters can be used for the response evaluation of VS following stereotactic RT.

Local experience with radiosurgery for vestibular schwannomas and recommendations for management

<p><b>INTRODUCTION</b>There are many treatment options for vestibular schwannomas (VSs), including radiosurgery. Previous studies have shown good outcomes for smaller tumours. We report the results of a seven-year cohort of patients with VS who were treated at our centre using a linear accelerator-based stereotactic radiosurgery system.</p><p><b>METHODS</b>We retrospectively reviewed the case notes and magnetic resonance (MR) images of patients with VS treated with radiosurgery. Treatment was administered as either a single 13 Gy session or 25 Gy in five sessions. At our centre, only larger or higher Koos grade VSs, were routinely treated with hypofractionated radiosurgery. Tumour response and hearing were assessed using RECIST criteria and Gardner-Robertson scale, respectively. Other toxicities were assessed using physical examination and history-taking. Freedom from radiological progression was estimated with the Kaplan-Meier method.</p><p><b>RESULTS</b>46 patients received single-fraction radiosurgery and 31 received hypofractionated radiosurgery. Median follow-up duration was 40.6 months. 29 patients had prior surgery to remove the tumour (median size 1.68 cm3). One patient who had symptomatic increase in tumour size (> 20% in largest diameter) was treated conservatively and subsequently showed stable disease on MR imaging. Progression-free survival was 98.7%. Another patient had symptomatic oedema requiring ventriculoperitoneal shunt insertion. 11 patients had serviceable hearing before radiotherapy and 72.7% of them retained useful hearing (20.1 dB mean decline in pure tone average). Facial and trigeminal nerve functions and sense of equilibrium were preserved in > 90% of patients.</p><p><b>CONCLUSION</b>Radiosurgery is effective and safe for small VSs or as an adjunct therapy after microsurgery.</p>

Morbidity Rate of the Retrosigmoid versus Translabyrinthine Approach for Vestibular Schwannoma Resection

BACKGROUND AND OBJECTIVES: Controversy related to the choice of surgical approach for vestibular schwannoma (VS) resection remains. Whether the retrosigmoid (RS) or translabyrinthine (TL) approach should be performed is a matter of debate. In the context of a lack of clear evidence favoring one approach, we conducted a retrospective study to compare the morbidity rate of both surgical approaches. SUBJECTS AND METHODS: 168 patients underwent surgical treatment (2007-2013) for VS at our tertiary care center. There were no exclusion criteria. Patients were separated into two groups according to the surgical approach: TL group and RS group. Signs and symptoms including ataxia, headache, tinnitus, vertigo and cranial nerve injuries were recorded pre- and postoperatively. Surgical complications were analyzed. Perioperative facial nerve function was measured according to House-Brackmann grading system. RESULTS: Tumor resection was similar in both groups. Facial paresis was significantly greater in RS group patients preoperatively, in the immediate postoperative period and at one year follow-up (p < 0.05). A constant difference was found between both groups at all three periods (p=0.016). The evolution of proportion was not found to be different between both groups (p=0.942), revealing a similar rate of surgically related facial paresis. Higher rate of ataxic gait (p=0.019), tinnitus (p=0.039) and cranial nerve injuries (p=0.016) was found in RS group patients. The incidence of headache, vertigo, vascular complications, cerebrospinal fluid leak and meningitis was similar in both groups. No reported mortality in this series. CONCLUSIONS: Both approaches seem similar in terms of resection efficacy. However, according to our analysis, the TL approach is less morbid. Thus, for VS in which hearing preservation is not considered, TL approach is preferable.

Schwannoma gigante del ángulo pontocerebeloso / Giant Schwannoma of the cerebellopontine angle

Los Schwannomas del acústico son tumores benignos de crecimiento lento de la división superior del nervio vestibular, con una incidencia de 1,9 por cada 100.000 habitantes. En la actualidad, la tecnología de la neuroimagen en conjunto con la exploración audiológica clínica e instrumentada permiten el diagnóstico en estadios tempranos e incluso como hallazgo clínico, por tal motivo solo el 6 % a nivel mundial se cataloga como tumor grande al momento del diagnóstico. Se presenta el caso de una mujer de 16 años con cefalea, mareo, vómito, hipoacusia, parálisis facial y diadococinesia, por lo que se realizaron estudios de neuroimagen en los que se evidenció masa ocupativa a nivel ángulo pontocerebeloso; por las dimensiones se cataloga según la clasificación de Koss como estadio IV. Se confirmó diagnóstico mediante estudio histopatológico.

Acoustic schwannomas are benign tumors of slow growth in the top division of the vestibular nerve, with an incidence of 1.9 per 100,000 inhabitants. Currently, imaging technology together with the clinical examination audiological and implemented allow diagnosis at an early stage and even as a clinical finding, on that ground only 6 % worldwide are categorized as large tumor at diagnosis. For a woman of 16 with headache, dizziness , vomiting , hearing loss, facial paralysis and diadochokinesia occurs, so neuroimaging studies in which a space- occupying mass level cerebellopontine angle were made evident ; by the dimensions it is classified as classified as stage IV Koss . Diagnosis was confirmed by histopathology.

Concomitant Occurrence of Vestibular Schwannoma and Epidermoid Cyst in Neurofibromatosis: Case description and Review of the Literature

We report a case of a 16-year-old female patient harboring neurofibromatosis type 2 who presented with bilateral hearing impairment, which was on the left side, as well as facial paresis (House-Brackmann grade III) and ataxic gait. A magnetic resonance imaging (MRI) exam evidenced bilateral lesions in the cerebellopontine angles (CPAs) with extension into the internal acoustic meatus, and an additional lesion in the right CPA with radiological characteristics of an epidermoid cyst. The patient was submitted to microsurgical resection, confirming a collision of a vestibular schwannoma and an epidermoid cyst in the right CPA. In the present case report, we describe the first case reported in the literature with preoperative diagnostic work-up, intraoperative findings, postoperative course of the patient, as well as a detailed literature review of these specific coinciding pathologies, denoting the importance of further genomic studies regarding multiple central nervous system (CNS) lesions.

Relatamos o caso de uma paciente de 16 anos de idade com neurofibromatose tipo II com deficiência auditiva bilateral, pior no ouvido esquerdo, assim como paresia facial (HouseBrackmann grau III) e ataxia. Estudo de ressonância magnética comprovou lesão bilateral nos ângulos cerebelopontinos (ACPs) com extensão ao meato acústico interno, e uma lesão adicional no ACP direito com características radiológicas de um cisto epidermoide. A paciente foi submetida a ressecção microcirúrgica, confirmando a colisão de um schwannoma vestibular com um cisto epidermoide no ACP direito. No presente estudo, descrevemos o primeiro caso relatado na literatura com trabalho diagnóstico pré-operatório, resultados intraoperatórios, evolução da paciente no pós-operatório, assim como revisão detalhada da literatura específica sobre essas patologias, demonstrando a importância de mais estudos genômicos sobre as múltiplas lesões do sistema nervoso central (SNC).

Trapped Fourth Ventricle in Recurrent Acoustic Schwannoma - A Rarity

Fourth ventricular dilatation is usually seen along with tri-ventriculomegaly in patients with communicating hydrocephalus. Isolated fourth ventricular dilatation is uncommon, especially as a sequelae following infective or post hemorrhagic communicating hydrocephalus. Communicating hydrocephalus is reported in vestibular schwannoma with an incidence of 3.7 to 23.5%, but 4th ventricular dilatation following its treatment has not been reported in the literature. We report a novel case of isolated fourth ventricular obstruction following surgery for recurrent vestibular schwannoma and ventriculoperitoneal shunt placement for communicating hydrocephalus. Management strategies range from endoscopic procedures to ventricular shunt placement. We describe the surgical technique for the placement of a fourth ventricular shunt with the use of a Y connector.

Em pacientes com hidrocefalia comunicante, a dilatação do quarto ventrículo é normalmente vista acompanhada de triventriculomegalia. A dilatação do quarto ventrículo isolado é incomum, especialmente como uma sequela subsequente à hidrocefalia comunicante infecciosa ou hemorrágica. Hidrocefalia comunicante é relatada em schwannoma vestibular com incidência de 3,7 a 23,5%, mas a dilatação do quarto ventrículo subsequente ao tratamento não foi encontrada na literatura. Relatamos caso de obstrução do quarto ventrículo isolado subsequente a cirurgia para schwannoma vestibular recorrente e shunt ventriculoperitoneal para hidrocefalia comunicante. As estratégias de manejo variam de procedimentos endoscópicos a shunt ventricular. Descrevemos técnica cirúrgica para alocação de shunt do quarto ventrículo com o uso de conector Y.

Long-Term Results of CyberKnife Radiosurgery for Vestibular Schwannoma / 대한평형의학회지

OBJECTIVE: Since the 2000s, CyberKnife radiosurgery (CKRS) is either a primary or an adjunct management approach used to treat patients with vestibular schwannoma (VS). The goals of CKRS are prevention of tumor growth, preservation of cranial nerve function and prevention of new neurologic deficiencies. The aim of this study was to assess the efficacy and safety of CKRS, in terms of tumor control, hearing preservation, and complications. METHODS: Forty patients with VS underwent CKRS as a treatment modality for from January 2010 to February 2016. The long term results of 32 patients were evaluated who received CKRS as primary treatment. 8 patients presented with previously performed surgical resection. Information related to clinical history, Brain MRI and outcomes of patients with VS collected retrospectively by reviewing patient's chart and telephone survey. RESULTS: The mean tumor volume was 3.3 cm³ and the mean follow-up was 41 months. The most recent follow-up showed that tumor size decreased in 17 patients (42.5%), displayed no change in 19 patients (47.5%), and increased in 4 patients (10%). Progression-free survival rates after CKRS at 1, 3, and 5 years were 95%, 90%, and 90%. After CKRS, 13 patients experienced hearing degradation. The overall rate of preservation of serviceable hearing at the long-term follow-up was 60%. Vertigo, ataxia, and headache were improved after CKRS compared with pretreated status. But, facial weakness, trigeminal nerve neuropathy, and tinnitus were worsen. CONCLUSION: CKRS provide an excellent tumor control rate and a comparable hearing preservation rate in VS patients. Also CKRS is associated with low rate of cranial neuropathy, other complications.

Simultaneous Translabyrinthine Tumor Removal and Cochlear Implantation in Vestibular Schwannoma Patients

Refinement of surgical techniques has allowed hearing preservation after tumor resection to be prioritized. Moreover, restoration of hearing after tumor removal can be attempted in patients with bilateral vestibular schwannomas or those with a schwannoma in the only-hearing ear. Cochlear implantation (CI) has emerged as a proper method of acoustic rehabilitation, provided that the cochlear nerve remains intact. Studies of electrical promontory stimulation in patients after vestibular schwannoma resection have demonstrated favorable results. We describe herein two cases of hearing rehabilitation via CI implemented at the time of vestibular schwannoma resection. Tumors were totally removed, and cochlear implant electrodes were successfully inserted in both cases. Also, post operative CI-aided hearing showed improved results.

Hearing Restoration in Neurofibromatosis Type II Patients

Patients with neurofibromatosis type II will eventually succumb to bilateral deafness. For patients with hearing loss, modern medical science technology can provide efficient hearing restoration through a number of various methods. In this article, several hearing restoration methods for patients with neurofibromatosis type II are introduced.

Vestibular Schwannoma Atypically Invading Temporal Bone

Vestibular schwannoma (VS) usually present the widening of internal auditory canal (IAC), and these bony changes are typically limited to IAC, not extend to temporal bone. Temporal bone invasion by VS is extremely rare. We report 51-year-old man who revealed temporal bone destruction beyond IAC by unilateral VS. The bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen. On histopathologic examination, the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature. Facial nerve was severely compressed and distorted by tumor, which unevenly eroded temporal bone in surgical field. Vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction. Early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone.