Technical aspects of fourth ventricle ependymomas in adults: how I do it.

BACKGROUND: Ependymomas in the fourth ventricle in adults are rare entity. Surgical treatment of adult ependymomas is the only treatment modality since no other effective alternative is available. Radical resection often means cure but it is hindered by the nature and location of the lesion. METHODS: Technical aspects of the fourth ventricle ependymoma surgery in adults are discussed. Anatomy of the area is provided with the step-by-step surgical algorithm. CONCLUSION: Radical resection of low-grade ependymoma with a detailed understanding of the anatomy in this area is vital considering the high effectiveness of the treatment and its excellent prognosis.

A case-based review on the neuroendoscopic management of intraventricular and subarachnoid basal neurocysticercosis.

OBJECTIVE: Extraparenchymal localization of neurocysticercosis (NCC) is rare in non-endemic areas. A case of mixed (intraventricular, IV, and subarachnoid basal, SAB) NCC was surgically treated using the neuroendoscope and a systematic review of the literature was performed with the aim to analyze the use of this instrument in the management of the extraparenchymal forms of the parasitic disease. MATERIALS AND METHODS: Medline and Embase databases were searched for studies where the neuroendoscope was used for the management of IV/SAB NCC cysts, either for the cerebrospinal fluid diversion or cyst removal. Cyst location, complete removal, cyst breakage during removal, intraoperative and postoperative complications, administration of antihelmintic therapy, outcome and follow-up period were extracted from the articles. RESULTS: 281 patients were treated by means of the neuroendoscope. 254 patients who were described in retrospective cohort studies, came all from endemic areas, with no significant difference between sexes. Mean age at surgery was 30.7 years. Of all cysts reported in retrospective studies, 37.9% were located in the fourth ventricle. An attempt of cyst removal was described in the 84.6% of cases and an endoscopic third ventriculostomy was performed in another 76.4%. A small number of complications were reported intraoperatively (9.1%) obtaining, but a good recovery was achieved at follow-up. Only 17 ventriculoperitoneal shunts were placed after the first procedure, defining a low risk of postoperative hydrocephalus even in case of partial cyst removal. CONCLUSION: Neuroendoscopic removal of an extraparenchymal NCC cyst is a safe procedure that should be preferred for lateral and third ventricle localization and, in a specialized centre, even for a localization in the fourth ventricle if feasible. It is also efficient because of the possibility of performing an internal CSF diversion concomitantly to cyst removal, avoiding the complication registered with VPS. The need for cysticidal treatment after surgery should be addressed in a prospective study.

Telovelar vs. Transvermian approach for the fourth ventricle tumors: A systematic review and meta-analysis.

BACKGROUND: Tumors in the fourth ventricle can be critical due to the small size of the fourth ventricle, which causes symptoms to be detected even in the presence of lesser mass effects. A proper surgical approach to the fourth ventricle poses challenges due to its deep location and proximity to vital compartments within the brainstem. The two commonly used approaches to these tumors are the transvermian and telovelar approaches. METHODS: A comprehensive systematic study was conducted based on a literature search of the databases. All case controls, cohorts, and case series including patients with fourth ventricle tumors, who were operated on with either telovelar or transvermian approaches were considered eligible. The evaluated outcomes were comparative postoperative complications of the telovelar vs. transvermian approach. After screening and data extraction, a meta-analysis was performed whenever adequate quantitative data were available. RESULTS: Seven studies with a total number of 848 patients, discussed both telovelar and transvermian approaches, with comparative reporting of outcomes in each group. Postoperative outcomes including cranial nerve deficit, mutism, diplopia, CSF leak, need for CSF diversion, and postoperative gait disturbance were not significantly different between telovelar and transvermian approaches. CONCLUSION: Postoperative complications were not significantly different between telovelar and transvermian approaches. Moreover, it could be proposed that such complications would be more likely to be a multifactorial matter concerning the patient's clinical condition, tumor characteristics, and surgeon's experience, rather than the surgical approach alone.

Subependymomas of the fourth ventricle: To operate or not to operate?

BACKGROUND: There is a paucity of literature regarding the clinical characteristics and management of subependymomas of the fourth ventricle due to their rarity. Here, we describe the operative and non-operative management and outcomes of patients with such tumors. METHODS: This retrospective single-institution case series was gathered after Institutional Review Board (IRB) approval. Patients diagnosed with a subependymoma of the fourth ventricle between 1993 and 2021 were identified. Clinical, radiology and pathology reports along with magnetic resonance imaging (MRI) images were reviewed. RESULTS: Patients identified (n = 20), showed a male predominance (n = 14). They underwent surgery (n = 9) with resection and histopathological confirmation of subependymoma or were followed with imaging surveillance (n = 11). The median age at diagnosis was 51.5 years. Median tumor volume for the operative cohort was 8.64 cm3 and median length of follow-up was 65.8 months. Median tumor volume for the non-operative cohort was 0.96 cm3 and median length of follow-up was 78 months. No tumor recurrence post-resection was noted in the operative group, and no tumor growth from baseline was noted in the non-operative group. Most patients (89 %) in the operative group had symptoms at diagnosis, all of which improved post-resection. No patients were symptomatic in the non-operative group. CONCLUSIONS: Surgical resection is safe and is associated with alleviation of presenting symptoms in patients with large tumors. Observation and routine surveillance are warranted for smaller, asymptomatic tumors.

Transpeduncular Approach for Resection of Ruptured Fourth Ventricular Arteriovenous Malformation.

The lateral recess of the fourth ventricle can be challenging to access surgically. We present a case of a previously ruptured arteriovenous malformation (AVM) of the fourth ventricle that was surgically resected via a retrosigmoid craniotomy and transinferior cerebellar peduncular approach. The patient is a 54-year-old female who experienced an intraventricular hemorrhage several months before presentation to our institution. Imaging studies suggested the presence of an AVM located in the right lateral recess of the fourth ventricle. After discussion of all possible treatment options, the patient elected to proceed with surgical resection. To access the lesion, we performed a retrosigmoid craniotomy and entered into the fourth ventricle via s small incision in the inferior cerebellar peduncle. Postoperative imaging demonstrated complete removal of the AVM nidus. Video 1 demonstrates this approach and discusses the anatomic landmarks used to guide resection.

Intraoperative Neurophysiologic Monitoring in Predicting Dysphagia After Brainstem and Fourth Ventricle Surgery.

OBJECTIVE: Dysphagia represents the main complication of posterior fossa neurosurgery. Adequate diagnosis of this complication is warranted to prevent untimely extubation with subsequent aspiration. Intraoperative neurophysiologic monitoring (IONM) modalities may be used for this purpose. However, it is not known which IONM modality may be significant for diagnosis. This study aimed to define the most significant IONM modality for dysphagia prognostication after posterior fossa neurosurgery. METHODS: The analysis included 46 patients (34 with tumors of the fourth ventricle and 12 with brainstem localization) who underwent surgical excision of the tumor. Neurologic symptoms before and after neurosurgery were noted and magnetic resonance imaging with the subsequent volume estimation of the removed mass was performed, followed by an IONM findings analysis (mapping of the nucleus of the caudal cranial nerves [CN] and corticobulbar motor-evoked potentials [CoMEP]). RESULTS: Aggravation of dysphagia was noted in 24% of the patients, more often in patients with tumor localization in the fourth ventricle (26%) than in those with brainstem mass lesions (16%). Mapping of the caudal cranial nerve nuclei did not correlate with the dysfunction of these structures. CoMEP was significantly associated with the neurologic state of the CN. The decrease in CoMEP is a significant prognostic factor for postoperative bulbar symptoms appearance or aggravation. CONCLUSIONS: Mapping the CN is an important identification tool. The CoMEP modality should be used intraoperatively to determine the functional state of the CN and predict postoperative dysphagia.

The different shapes of the fourth ventricle.

With a distinctive shape and surrounding anatomical structures, the fourth ventricle is located in the posterior cranial fossa. There are various pathologies, either developmental or acquired, that can present as a characteristic deformity of the fourth ventricle. Therefore, this paper will cover the anatomy of the fourth ventricle and correlate this to the various pathologies. The aim of this review is to improve the ability of the readers to recognise the change in shape and configuration of the fourth ventricle, enabling early detection of pathologies.

Rare Case of Hemorrhagic Atypical Choroid Plexus Papilloma of Fourth Ventricle in Adult: A Diagnostic Dilemma.

Choroid plexus papillomas are highly vascular tumors, and such tumors causing subarachnoid hemorrhage have been reported in literature. Similarly, few articles have reported atypical fourth ventricular choroid plexus tumors in adults. However, such an atypical tumor presenting with grossly hemorrhagic transformation without any acute symptoms could not be found in the literature.

Anatomical Implications of Chiari I and Basilar Invagination (Type B) in the IV Ventricle and Cisterna Magna.

OBJECTIVE: To analyze the anatomical changes of the IV ventricle and cisterna magna in the Chiari malformation I (CMI) and basilar invagination (type B). METHODS: This is a controlled study with 161 exams of magnetic resonance imaging (MRI) of adults grouped into control (n = 37), basilar invagination (BI; n = 31), Chiari malformation I (CMI; n = 37), and CMI+BI (n = 56). The MRIs were analyzed using the visualization software Osirix (Pixmeo, Bernex, Geneva, version 3.8.2). The morphometric variables were: distance from the obex to the McRae line; length of the IV ventricle floor; and the area and volume of the cisterna magna. The univariate ANOVA followed by Tukey's post-hoc test was applied to evaluate the difference between the groups. The difference between sexes was evaluated by the t test for each group. RESULTS: Alterations in the cisterna magna and IV ventricle were more evident only in the CMI and CMI+BI groups. For both sexes, the CMI and CMI+BI groups showed: a reduction in the CSF space (P < 0.001), cisterna magna with volume reduction (P < 0.001), low position of the obex (P < 0.001), and IV ventricle more elongated (male P = 0.007 and female P < 0.001). The BI group had no significant change in the analysis by sex. CONCLUSIONS: The CMI (isolated and associated with BI) showed a low obex position and elongation of the IV ventricle due to traction towards the spinal canal. The reduction of cisterna magna volume added to the occupation of the cerebellar tonsils can impact in the cerebrospinal fluid dynamics. The BI when isolated was not related to alterations in the parameters of cerebrospinal fluid spaces studied.

Predictors of postoperative complications and functional outcomes in pediatric patients with surgically treated fourth ventricle tumors.

BACKGROUND: Tumors of the fourth ventricle are frequently treated pathologies in pediatric neurosurgery. Data regarding predictors for permanent neurological deficits, long-term functional outcomes, cerebellar mutism (CM), the extent of resection (EOR), and oncological outcomes are scarce. We attempt to contribute to this topic with an analysis of our institutional cohort. METHODS: A retrospective single-center study of patients aged ≤ 19 years who underwent primary surgical resection of a fourth ventricular tumor over a 15-year period (2006-2021). Predictors analyzed included age, gender, surgical approach, anatomical pattern, tumor grade, EOR, tumor volume, and others as appropriate. RESULTS: One hundred six patients were included (64 males, mean age 7.3 years). The rate of permanent neurological deficit was 24.2%; lateral tumor extension (p = 0.036) and tumor volume greater than 38 cm3 (p = 0.020) were significant predictors. The presence of a deficit was the only significant predictor of reduced (less than 90) Lansky score (p = 0.005). CM occurred in 20.8% of patients and was influenced by medulloblastoma histology (p = 0.011), lateral tumor extension (p = 0.017), and male gender (p = 0.021). No significant difference between the transvermian and telovelar approach in the development of CM was detected (p = 0.478). No significant predictor was found for the EOR. EOR was not found to be a significant predictor of overall survival for both low-grade and high-grade tumors; however, gross total resection (GTR) was protective against tumor recurrence compared to near-total or subtotal resection (p < 0.001). In addition, survival was found to be better in older patients (≥ 7.0 years, p = 0.019). CONCLUSION: The overall rate of postoperative complications remains high due to the eloquent localization. Older patients (> 7 years) have been found to have better outcomes and prognosis. Achieving GTR whenever feasible and safe has been shown to be critical for tumor recurrence. CM was more common in patients with medulloblastoma and in patients with tumors extending through the foramen of Luschka. The telovelar approach uses a safe and anatomically sparing corridor; however, it has not been associated with a lower incidence of CM and neurological sequelae in our series, showing that each case should be assessed on an individual basis.

Intraventricular migration of fourth ventricular neurocysticercosis: an unusual complication during endoscopic surgery.

A boy in his middle childhood presented with intermittent episodes of headache with vomiting for 6 months. Plain CT of the head and MRI of the brain revealed fourth ventricular cysticercal cyst with acute obstructive hydrocephalus. Endoscopic excision of the cyst was done along with endoscopic third ventriculostomy and septostomy with external ventricular drain placement. Although we were able to decompress the cysticercal cyst, unfortunately, the cyst got slipped from the grasper leaving the grasped cyst wall in the tooth of the grasper. Through this case report, we want to highlight that such a complication could also happen during neuroendoscopic cysticercal cyst removal and how we dealt with it. Our patient was discharged neurologically intact and was symptom free on follow-up.

Staged Gamma Knife radiosurgery for a rosette-forming glioneuronal tumor of the fourth ventricle: a case report.

BACKGROUND: Rosette-forming glioneuronal tumor (RGNT) is a rare slow-growing neoplasm with mixed glial and neurocytic components. Surgical resection is the mainstay of treatment, whereas the role of adjuvant radiation therapies for residual or recurrent tumors has been poorly investigated. CASE PRESENTATION: We describe the case of a patient with a recurrent fourth ventricular RGNT who was treated with two-staged Gamma Knife radiosurgery (GKRS). GKRS was effective in controlling tumor growth and safe up to seven years from treatment. CONCLUSIONS: This case suggests that GKRS may be a safe and effective treatment for patients with recurrent or residual RGNT.

Neuronavigated endoscopic aqueductoplasty with panventricular stent plus septostomy for isolated fourth ventricle in complex hydrocephalus and syringomyelia associated with myelomeningocele: how I do it.

BACKGROUND: Isolated fourth ventricle (IFV) is a challenging entity to manage. In recent years, endoscopic treatment for aqueductoplasty has been on the rise. However, in patients with complex hydrocephalus and distorted ventricular system, its implementation can be complex. METHODS: We present a 3-year-old patient with myelomeningocele and postnatal hydrocephalus treated by ventriculoperitoneal shunt. In follow-up, a progressive IFV and isolated lateral ventricle with symptoms of the posterior fossa developed. An endoscopic aqueductoplasty (EA) with panventricular stent plus septostomy guided with neuronavigation was decided due to the complexity of the ventricular system. CONCLUSION: In IFV associated with complex hydrocephalus with distortion of the ventricular system, navigation can be of great help for planning and as a guide for performing EA.

Trapped Fourth Ventricle: Pathophysiology, History and Treatment Strategies.

Trapped fourth ventricle is a clinic-radiological entity characterised by progressive neurological symptoms due to enlargement and dilatation of fourth ventricle secondary to obstruction to its outflow. There are several causative mechanisms for the development of trapped fourth ventricle, including previous haemorrhage, infection or inflammatory processes. However, this condition is most commonly observed in ex preterm paediatric patients shunted for a post-haemorrhagic or post-infective hydrocephalus. Until the introduction of endoscopic aqueductoplasty and stent placement, treatment of trapped fourth ventricle was associated with high rates of reoperation and complications resulting in morbidity. With the advent of new endoscopic techniques, supratentorial and infratentorial approaches for aqueductoplasty and stent insertion have revolutionised the treatment of trapped fourth ventricle. Fourth ventricular fenestration and direct shunting remain viable options in cases where aqueduct anatomy and length of obstruction is not surgically favourable for endoscopic approaches. In this book chapter, we explore the background, historical developments,$ and surgical treatment strategies in the management of this challenging condition.

Impact of choroid plexus size in prenatal diagnosis of normal and abnormal closure of fourth ventricle.

OBJECTIVE: To assess the role of the choroid plexus (CP) of the fourth ventricle (4V) in fetuses with an open 4V and a normal cerebellar vermis. METHODS: Two groups of patients were recruited in two fetal medicine referral centers. The prospectively collected control group included singleton pregnancies with a normal sonographic examination after first-trimester combined screening for chromosomal abnormalities and normal outcome, recruited in the period between 2019 and 2022. The study group was selected retrospectively by searching our databases to identify all cases with an isolated open 4V and normal anatomy and size of the cerebellar vermis. The inclusion criteria of the study group were: (1) gestational age between 20 and 22 weeks; (2) a brainstem-vermis angle ≥ 18° in the midsagittal plane with an otherwise normal cerebellum and vermis; (3) 4V-CP visible and seen separately from the vermis; (4) absence of other intra- and extracranial anomalies; and (5) available prenatal and/or postnatal magnetic resonance imaging (MRI) data. RESULTS: In 169 cases of the control group, the 4V-CP was seen separately from the cerebellar vermis and was noticed to progressively fill the space caudal to the 4V, between the vermis and brainstem. From 12 to 22 weeks, the surface areas of the vermis and medial portion of the 4V-CP increased progressively with advancing gestation (P < 0.0001). Intra- and interobserver correlation analysis showed good reproducibility for the measurements. Among the cases with an open 4V and a normal vermis, it was retrospectively feasible to visualize the 4V-CP separately from the inferior part of the vermis in 41 fetuses. In five of these cases, the open 4V was due to a small CP. In all 41 fetuses, the diagnosis on MRI was isolated upward rotation of the cerebellar vermis, and no additional anomaly was found. CONCLUSIONS: Closure of the 4V is dependent on the 4V-CP and not only the cerebellar vermis. In fact, a small CP may represent another cause of an open 4V. Therefore, separate visualization of the 4V-CP and cerebellar vermis is crucial to improve discrimination between the different causes of an open 4V at the anomaly scan and its clinical implications. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Combined telovelar posterolateral (far lateral) approach for the resection of a large posterior fossa ependymoma: how I do it.

BACKGROUND: Ependymomas are glial cell tumors whose recommended treatment, according to the recent European guidelines, is surgical. Patient outcomes, in terms of progression-free survival and overall survival, are strongly related to the extent of resection. However, in some cases, critical locations and/or large dimensions could make a gross total resection challenging. In this article, we describe the surgical anatomy and technique of a combined telovelar-posterolateral approach for the resection of a giant posterior fossa ependymoma. METHODS: A 24-year-old patient who presented to our institution complaining of a 3-month history of headache, vertigo, and imbalance. Preoperative MRI scans showed a large mass within the fourth ventricle, extending towards the left cerebellopontine angle and perimedullary space through the homolateral Luschka foramen. Surgical treatment was proposed with the aims of releasing the preoperative symptoms, obtaining the tumor's histopathological and molecular definition, and preventing any future neurological deterioration. The patient gave his written consent for surgery and consented to the publication of his images. A combined telovelar-posterolateral approach was then performed to maximize the tumor's exposure and resection. Surgical technique and anatomical exposure have been extensively described, and a 2-dimensional operative video has been included. RESULTS: The postoperative MRI scan demonstrated an almost complete resection of the lesion, with only a millimetric tumor remnant infiltrating the uppermost portion of the inferior medullary velum. Histo-molecular analysis revealed a grade 2 ependymoma. The patient was discharged home neurologically intact. CONCLUSIONS: The combined telovelar-posterolateral approach allowed to achieve a near total resection of a giant multicompartimental mass within the posterior fossa in a single surgical stage.

The Roof of the 4th Ventricle Seen From Inside: Endoscopic Anatomic Illustration-A Case Series.

BACKGROUND: The anatomy of the roof of the fourth ventricle has been illustrated in many laboratory investigations, but in vivo reports of the roof anatomy and its variants are still lacking. OBJECTIVE: To describe the topographical anatomy of the roof of the fourth ventricle explored through a transaqueductal approach that overcomes cerebrospinal fluid depletion, displaying in vivo anatomic images possibly quite close to normal physiological conditions. METHODS: We critically reviewed the intraoperative video recordings of our 838 neuroendoscopic procedures, selecting 27 cases of transaqueductal navigation that provided good quality image details of the anatomy of the roof of the fourth ventricle. Twenty-six patients affected by different forms of hydrocephalus were therefore categorized into three groups: Group A: blockage of the aqueduct-aqueductoplasty, Group B: communicating hydrocephalus, and Group C: tetraventricular obstructive hydrocephalus. RESULTS: Group A has shown what the roof of a normal fourth ventricle really looks like albeit the structures seemed overcrowded because of the narrow space. Images from groups B and C paradoxically allowed a more distinct identification of the roof structures flattened by ventricular dilation, making them more comparable with the topography traced on the laboratory microsurgical studies. CONCLUSION: Endoscopic in vivo videos and images provided a novel anatomic view and an in vivo redefinition of the real topography of the roof of the fourth ventricle. The relevant role of cerebrospinal fluid was defined and outlined, as well as the effects of hydrocephalic dilation on some structures on the roof of the fourth ventricle.