Guía de práctica clínica sobre el manejo del schwannoma vestibular / Clinical practice guideline on the management of vestibular schwannoma

Introducción: El schwannoma vestibular (SV) es el tumor más frecuente del ángulo pontocerebeloso. La mayor accesibilidad a las pruebas radiológicas ha incrementado su diagnóstico. Teniendo en cuenta las características del tumor, la clínica y la edad del paciente se han propuesto tres estrategias terapéuticas, observación, cirugía o radioterapia. La elección de la más adecuada para cada paciente es un motivo de controversia frecuente. Material y métodos: El presente trabajo incluye una revisión exhaustiva sobre cuestiones relativas al SV que pueden servir de guía clínica en el manejo de pacientes con estas lesiones. La presentación se ha orientado en forma de preguntas que el clínico se hace habitualmente y las respuestas están redactadas y/o revisadas por un panel de expertos nacionales e internacionales consultados por la Comisión de Otología de la SEORL-CCC. Resultados: Se ha elaborado un listado con los 13 bloques temáticos más controvertidos sobre el manejo del SV en forma de 50 preguntas y se han buscado las respuestas a todas ellas mediante una revisión sistemática de la literatura (artículos publicados en PubMed y Cochrane Library entre 1992 y 2023 sobre cada bloque temático). Treinta y tres expertos, liderados por la Comisión de Otología de la SEORL-CCC, han analizado y discutido todas las respuestas. En el Anexo 1 pueden encontrarse 14 preguntas adicionales divididas en cuatro bloques temáticos. Conclusiones: Esta guía de práctica clínica sobre el manejo del SV ofrece respuestas consensuadas a las preguntas más habituales que se plantean sobre este tumor. La ausencia de suficientes estudios prospectivos hace que los niveles de evidencia sobre el tema sean en general medios o bajos. Este hecho incrementa el interés de este tipo de guías de práctica clínica elaboradas por expertos.(AU)

IntroductionVestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. Material and methods: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. Results: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. Conclusions: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.(AU)

Arachnoid Cyst of the Cerebellopontine Angle: A Systematic Literature Review.

OBJECTIVE: The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date. METHODS: A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed. RESULTS: A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years]. CONCLUSIONS: Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.

Cerebellopontine angle tumor presenting as acute audiovestibular syndrome.

BACKGROUND: Acute audiovestibular deficits may be a harbinger of vestibular schwannoma (VS). OBJECTIVE: To investigate clinical and laboratory features of 25 consecutive patients with VS presenting with acute audiovestibular deficits. METHODS: A symptomatic combination of acute audiovestibular deficits was investigated. Audiometric and vestibular function tests, and internal auditory canal magnetic resonance imaging (IAC MRI) results were evaluated. RESULTS: Varying combinations of symptoms may develop in VS patients with acute audiovestibular deficits, of whom sudden hearing loss (HL) without acute vertigo or acute facial nerve palsy (FNP) was most common. The most common audiometric configuration was high-tone hearing loss, and no patient showed low-tone hearing loss. IAC MRI demonstrated that the tumor had an intracanalicular portion and attachment to the bony IAC wall in all patients and widened the IAC wall in some patients. CONCLUSION: Different symptomatic combinations of acute audiovestibular deficits may develop in patients with VS. Awareness about the possibility of VS as a cause of sudden HL, acute vertigo, and acute FNP, as well as subsequent IAC MRI scanning is vital to earlier diagnosis of VS in these patients.

The cerebellopontine angle cistern volumetric differences in trigeminal neuralgia patients with and without vertebrobasilar compression: a case-matched study.

Previous studies have indicated that the small cerebellopontine angle (CPA) cistern plays a role in the pathogenesis of trigeminal neuralgia (TN), but they are likely not involved in TN associated with vertebrobasilar artery (VBA) compression because of its rarity. Forty-four patients with VBA-associated TN and 44 age-, sex-, and hypertension-matched TN patients without VBA compression (non-VBA-associated) were included. All patients underwent high-resolution MRI. The CPA cistern volumes were measured bilaterally. The presence of vertebrobasilar dolichoectasia (VBD) and laterality of the vertebrobasilar junction (VBJ) were observed. The CPA cistern volume on the affected side was smaller than the unaffected side (714.4 ± 372.8 vs 890.2 ± 462.2 mm3, p < 0.001) in non-VBA-associated TN patients, while VBA-associated TN patients show a larger CPA cistern on the affected side than the unffected side (1107.0 ± 500.5 vs 845.3 ± 314.8 mm3, p < 0.001). The prevalence of VBD was higher in patients with VBA-associated TN than in matched non-VBA-associated TN patients (90.9% vs 4.5%, p < 0.001). A positive correlation between the laterality of VBJ and the affected side was found in the VBA-associated TN group (p < 0.0001). Large CPA cistern may be a neuroradiological feature of VBA-associated TN, and most of the VBA-associated TN is accompanied by VBD. The presence of VBD and the lateral shift of VBJ may expand the CPA cistern by squeezing the surrounding tissue on the affected side and also increase the chance of VBA compression on the trigeminal nerve, resulting in the genesis of VBA-associated TN.

Upfront Radiosurgery vs a Wait-and-Scan Approach for Small- or Medium-Sized Vestibular Schwannoma: The V-REX Randomized Clinical Trial.

Importance: Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically. Objective: To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma. Design, Setting, and Participants: Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021. Interventions: Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires. Main Outcomes and Measures: The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications. Results: Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed. Conclusion and relevance: Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed. Trial Registration: ClinicalTrials.gov Identifier: NCT02249572.

Bruns Nystagmus in Cerebellopontine Angle Hemangioblastoma.

Bruns nystagmus is a form of jerk nystagmus that has a localizing value in cerebellopontine angle (CPA) tumors. Hemangioblastomas involving the CPA is rare. A case of a 57-year-old male presented with Bruns nystagmus which led to the discovery of a large CPA hemangioblastoma is described. The nystagmus was compatible with the laterality of the tumor. High quality video of Bruns nystagmus was recorded.

Diluted intracisternal papaverine for microvascular protection of cranial nerves during vestibular schwannoma and cerebello-pontine angle surgery. Commentary and review of the literature.

Vasospasm after resection of skull base tumors is a rare complication that often produces relevant ischemic sequelae. This review of the literature reports a number of published experiences that can help determine the potential causes of vasospasm after cerebello-pontine angle (CPA) tumor and -in particular-vestibular schwannoma (VS) resection, the ways to prevent it, and the methods to obtain the correct diagnosis. The cause appears to be multifactorial and the surgical approach may contribute to the pathogenesis of vasospasm. Neurosurgeons must pay attention to detect possible vasospasm at an early stage of cerebello-pontine. Cerebral blood flow measurement and transcranial Doppler are useful monitoring tools. Intra-operative prevention of vasospasm during CPA tumor resection with papaverine hydrochloride (PPV) seems to play a relevant role. In particular, PPV is a direct-acting vasodilator used to manage vasospasm during various neurosurgical operations. There is large uncertainty about intracisternal PPV dose-related efficacy and side effects. Dilution of PPV in saline prior to application is recommended to avoid complications. In our experience, in line with the literature, we use a pure PPV without excipients 60 mg/2 ml diluted in 20 cc of 0,9% saline solution (0,3%) to prevent Hearing Loss during Posterior Fossa Microvascular Decompression for Typical Trigeminal Neuralgia and other cranial nerves potentially involved during VS and other CPA tumor resection. The aim of this commentary is to analyze and discuss the role of diluted intracisternal PPV for microvascular protection of cranial nerves during CPA tumor surgery.

Radiological presence of vascular loops in the cerebellopontine angle region in patients with unilateral Ménière's disease.

OBJECTIVE: The relationship between vascular compression of the vestibulocochlear nerve and audio-vestibular symptoms remains controversial. We aimed to examine the radiological features of vascular loops signs in cerebellopontine angle (CPA) and internal auditory canal (IAC) in patients with unilateral Ménière's disease (MD). METHODS: One hundred and thirty-seven patients with unilateral definite MD and 69 control subjects (138 ears) were enrolled. All subjects received magnetic resonance imaging of CPA-IAC. The configuration of vascular loops in CPA-IAC, based on the Kazawa classification system, from MD-affected, non-affected and control ears were compared. The associations between imaging findings and Ménière's stage, electrocochleogram (EcochG) and caloric test were analyzed. RESULTS: (1) Among the MD-affected ears, 6 cases (4.4%) were classified as Kazawa type IA, 27 cases (19.7%) as IB, 60 cases (43.8%) as IIA, and 44 cases (32.1%) as IIB. No significant interaural difference in the distribution of Kazawa's types was found ([Formula: see text] = 4.737, p = 0.578) in unilateral MD patients. (2) The distribution of Kazawa's types were not significantly different between the MD-affected ears and the control subjects ([Formula: see text] = 2.876, p = 0.411). (3) No relationship was found between Kazawa staging of the MD-affected ear and Ménière's stage (H = 2.679, p = 0.444), EcochG ([Formula: see text] = 0.827, p = 0.867) and caloric test ([Formula: see text] = 4.116, p = 0.248). CONCLUSIONS: In patients with unilateral MD, the configuration of vascular loops in CPA-IAC region, measured by Kazawa criteria, did not correlate with the laterality, clinical stage, the results of EcochG and caloric test, suggesting that vascular loops may be natural anatomical variations for patients with MD.

Solitary Sclerotic Fibroma of Right Cerebellopontine Angle.

Sclerotic fibroma (storiform collagenoma) is a fibrotic tumor that occurs mainly in patients with Cowden syndrome, but it can also occur in isolation, as detailed in previous reports. Here we present a case of a solitary sclerotic fibroma in cerebellopontine angle. Brain magnetic resonance imaging revealed a lesion showing hypointense signal on both T1 and T2. The lesion was not enhanced after administering gadolinium. The tumor was removed integrally by surgery.

Fascicular-Sparing Technique for Resection of Cerebellopontine Angle Facial Nerve Schwannoma.

Surgery of cerebellopontine angle (CPA) facial nerve schwannoma (FNS) in patients with good facial nerve function is a challenge.1-10Video 1 highlights the fascicular-sparing technique for resection of a CPA FNS. A 41-year-old male patient symptomatic with persistent headaches and tinnitus underwent a retrosigmoid approach for a right cystic CPA tumor, presumed vestibular schwannoma. Intraoperatively, the facial nerve was identified as fine multiple strands splayed around the perimetry of the tumor, which elicited a motor response at a low threshold stimulation. This finding led to the intraoperative diagnosis of FNS according to the reported criteria.5 Neuromonitoring-assisted fascicular-sparing resection technique was performed. It involved the gradual separation of the uninvolved nerve fibers using a fine-stimulating dissector at a threshold of 0.2 mA. Entry into the tumor was at a stimulation silent cyst. The tumor was debulked with preservation of the endoneurium and pulse irrigation hemostasis. A near-total resection was performed. The patient was discharged on the second postoperative day with a House-Brackman III facial nerve deficit. The deficit remained stable during the following annual follow-up visits. Resection of CPA FNS is indicated at the earliest sign of deficit. However, it might be encountered as masquerading at the surgery of an acoustic tumor. The fascicular-sparing technique is critical in avoiding injuries to the endoneurium during the resection and with the ability to preserve function. The sparing of endoneurium avoids collagenization, fibrosis, and ischemia of the nerve, which are known to be the pathologic substrate of worse functional outcomes.

Intracochlear signal in FIESTA-C and hearings of patients with cerebellopontine angle schwannoma.

BACKGROUND: Hearing loss in patients with cerebellopontine angle (CPA) schwannoma, is thought to be caused by the damage to the cochlea and the cochlear nerve. AIM: This study aimed to examine the relationships between the intracochlear signal in magnetic resonance imaging (MRI) and hearing in patients with CPA schwannoma. MATERIAL AND METHOD: In 79 patients with CPA schwannoma, we retrospectively examined the signal in the cochlea on the affected side was compared with that on the unaffected side to determine signal degradation in fast imaging reagents steady-state acquisition with cycle phases (FIESTA-C) MRI. For hearing evaluation, pure tone audiometry (PTA), speech audiometry, distortion product otoacoustic emissions (DPOAE), and auditory brainstem response (ABR) were used. For each parameter, we examined the differences between the groups with and without signal degradation. RESULTS: In the hearing test results, the I-wave latency of ABR was significantly longer in the group with signal degradation in FIESTA-C (1.84 ± 0.35 msec vs. 2.04 ± 0.37 msec, p = 0.048). There was no statistically significant difference in other tests. CONCLUSION: The MRI signal changes in the cochlear were related to the I-wave latency of ABR and reflected cochlear function. SIGNIFICANCE: We suggested the cochlear signal changes in CPA schwannoma patients related the hearing.

Analysis of Imaging Results for Semisitting Compared with Supine Positioning in the Retrosigmoid Approach for Resection of Cerebellopontine Angle Vestibular Schwannomas.

OBJECTIVE: To compare the completeness of resection of vestibular schwannomas using three-dimensional segmented volumetric analysis of pre- and postoperative magnetic resonance imaging (MRI) of patients undergoing supine and semisitting positioning for the retrosigmoid approach. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary medical center. PATIENTS: Patients with vestibular schwannomas undergoing surgical resection via the retrosigmoid approach. INTERVENTIONS: Tumor resection via the retrosigmoid approach with different patient positioning: standard supine versus semisitting. MAIN OUTCOME MEASURES: Preoperative versus postoperative three-dimensional segmented volumetric MRI analysis of vestibular schwannomas. RESULTS: A total of 43 patients (15 supine and 28 semisitting) underwent retrosigmoid craniotomy for resection of vestibular schwannomas. For the conventional supine and semisitting positioning, mean preoperative tumor volumes were 12.65 and 8.73 cm 3 ( p = 0.15), respectively. Postoperative mean tumor volumes for the supine and semisitting positions were 2.09 and 0.48 cm 3 ( p = 0.13), respectively. There were 11 cases of postoperative sigmoid sinus thrombosis, 3 in the conventional supine group and 8 in the semisitting groups, and there were 6 cases of postoperative cerebrospinal fluid leaks, all in the semisitting group. The mean House-Brackmann scores for the supine and semisitting groups were 2.9 and 2.3, respectively. There was no statistically significant difference between groups in the rates of these or any other postoperative complications. CONCLUSIONS: The semisitting position for the suboccipital retrosigmoid approach for vestibular schwannoma resection does not compromise the ability to adequately resect the tumor as seen by volumetric MRI results. Further studies are needed to establish the safety of this position compared with the traditional supine approach.

Cerebellopontine angle epidermoid with ipsilateral external ear atresia: an embryological association or a coincidence?

The cerebellopontine angle (CPA) is the most common site for intracranial epidermoids. They may have varied presentations viz facial pain, hemifacial spasms, headache and uncommonly features of raised intracranial pressure. Their association with external ear atresia has not been reported in literature to date. We present a very rare case of CPA epidermoid with ipsilateral external ear atresia.

Balloon compression, neuroendoscopy and electrophysiological monitoring-assisted retrosigmoid approach for resection of cholesteatoma in the cerebellopontine angle-Meckel's cave: a technical description.

We report a case of cholesteatoma that caused left facial pain with facial numbness. The tumour was located in the left cerebellopontine angle (CPA) and Meckel's cave. A balloon was first placed into Meckel's cave, and then, under electrophysiological monitoring, the tumour within the CPA cistern was resected via the retrosigmoid approach. The balloon was inflated in Meckel's cave to push the tumour out of Meckel's cave, and then, the tumour was completely removed under endoscopy. The symptoms, including pain and numbness, subsided after surgery.

How I do it: cerebellopontine angle epidermoid cyst removal with endoscopic assistance.

BACKGROUND: Epidermoid cyst in the cerebellopontine angle (CPA) can involve numerous critical structures. Endoscopic assistance following microscopic removal via a retrosigmoid approach can expand the surgical corridor, allowing for more complete resection. METHOD: We describe in a stepwise fashion the surgical steps for the microscopic removal of an epidermoid cyst of the CPA with endoscopic assistance. CONCLUSION: Endoscopically assisted microscopic removal for CPA epidermoid cysts provides wide access to the CPA and its adjacent structures and shows to be an effective option in selected cases.