Predicting the presence of 4th ventricular outlet obstruction in Chiari I Malformation.

INTRODUCTION: A subset of children with Chiari 1 malformation (CM-1) have a 4th ventricle arachnoid veil-a thin membrane covering the outlet of the 4th ventricle. Studies suggest that failure to disrupt this veil during posterior fossa decompression can reduce the likelihood of syringomyelia resolution. However, there is no reliable method for predicting the presence of the veil without direct surgical exploration. This study aims to evaluate the association between pre-operative symptoms, radiographic measurements, and the arachnoid veil. METHODS: A retrospective review of an institutional database of children evaluated for CM-I was conducted. For patients treated with surgery, operative notes were reviewed to determine if an arachnoid veil was present. Logistic regression was used to test for relationship of clinical variables and radiographic measurements with the presence of an arachnoid veil. RESULTS: Out of 997 children with CM-1, 226 surgical patients were included in the analysis after excluding those with inadequate documentation. An arachnoid veil was found in 23 patients (10.2%). Larger syrinx, spinal canal, and thecal sac diameters were significantly associated with the presence of a veil, with odds ratios of 1.23 (95% CI 1.2-1.48; p = 0.03), 1.27 (95% CI 1.02-1.59; p = 0.03), and 1.35 (95% CI 1.03-1.77; p = 0.03), respectively. No significant associations were found with any signs or symptoms. CONCLUSIONS: Arachnoid veil was present in 10% of cases. Radiographic measurements indicating larger syrinx size were the only variables found to be significantly associated with an arachnoid veil. Exploration of the 4th ventricular outlet is recommended for CM-I decompression in the setting of expansile syringomyelia.

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.

The telovelar approach for fourth ventricular tumors in children: is removal of the posterior arch of C1 necessary?

PURPOSE: Various surgical nuances of the telovelar approach have been suggested. The necessity of removing the posterior arch of C1 to accomplish optimal tumor exposure is still debated. Therefore, we report on our experience and technical details of the fourth ventricular tumor resection in a modified prone position without systematic removal of the posterior arch of C1. METHODS: A retrospective analysis of all pediatric patients, who underwent a fourth ventricular tumor resection in the modified prone position between 2012 and 2021, was performed. RESULTS: We identified 40 patients with a median age of 6 years and a M:F ratio of 25:15. A telovelar approach was performed in all cases. In 39/40 patients, the posterior arch of C1 was not removed. In the remaining patient, the reason for removing C1 was tumor extension below the level of C2 with ventral extension. Gross or near total resection could be achieved in 34/39 patients, and subtotal resection in 5/39 patients. In none of the patients, a limited exposure, sight of view, or range of motion caused by the posterior arch of C1 was encountered, necessitating an unplanned removal of the posterior arch of C1. Importantly, in none of the cases, the surgeon had the impression of a limited sight of view to the most rostral parts of the fourth ventricle, which necessitated a vermian incision. CONCLUSION: A telovelar approach without the removal of the posterior arch of C1 allows for an optimal exposure of the fourth ventricle provided that critical nuances in patient positioning are considered.

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.

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.

Experience in treating children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle and systematic review.

OBJECTIVE: To investigate the treatment plan and prognosis of children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle. METHODS: In this retrospective study, the clinical information of 10 consecutively collected children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle was analyzed. All 10 children underwent pontine tumour resection through a trans-cerebellomedullary fissure approach; 4 children underwent preoperative diffusion tensor imaging scans to determine the relationship between the tumour and facial nerve nucleus, and the other 6 children underwent intraoperative deep electroencephalography (EEG) tumour monitoring, in which the tumour electrical discharge activity of the tumour was recorded. A voxel distribution map was established to describe the distribution of the tumour location, and patient prognosis was evaluated through clinical and imaging follow-up. RESULTS: All 10 children achieved total tumour resection; 9 tumours were pathologically suggested to be ganglioglioma (WHO grade I), and 1 was a hamartoma. The symptoms of the original ocular dyskinesia and hemifacial spasm disappeared immediately after the operation. The children were followed up for 4-75 months, and none of the symptoms recurred; four cases with preoperative diffusion tensor imaging showed that the tumour was close to the facial nerve. Four in six intraoperative electrophysiological monitoring showed that the tumour had electrical discharge behaviour, and the tumour distribution map indicates a high density of tumour presence in the facial nerve nucleus and the nucleus of the abducens nerve. CONCLUSIONS: In paediatric patients, the facial symptoms are related to the location and abnormal electrical discharge of the tumour. There is no significant correlation between ocular dyskinesia and the location of the tumour. Conventional antiepileptic therapy for this disease is ineffective, and early surgical intervention for total tumour resection can achieve a clinical curative effect.

Successful Surgical Resection of an Ependymal Cyst in the Fourth Ventricle of a Dog.

Ependymal cysts represent congenital brain malformations rarely described in human medicine, where surgical resection is the treatment of choice. In veterinary medicine, only three cases have been previously reported, with one partially resected with surgery. A 6 yr old entire male American Staffordshire terrier was referred with a 4 mo history of incoordination and collapsing episodes with extensor rigidity. Neurological examination localized the lesion to the left central vestibular system and cerebellum. A brain computed tomography scan showed a hypoattenuating lesion with peripheral contrast enhancement in the fourth ventricle consistent with a cyst and secondary hydrocephalus. Treatment with prednisone was initiated, but despite an initial improvement, neurologic signs recurred and a suboccipital craniectomy to remove the cyst was performed. The cyst was first drained, and the capsule was carefully resected. The histopathological evaluation revealed a simple cubic to cylindrical epithelium with apical cilia and loose surrounding fibrillar tissue consistent with an intraventricular ependymal cyst. Four and a half years after surgery, the dog only shows short episodes of balance loss when turning abruptly but is otherwise neurologically normal. To the authors' knowledge, this is the first reported ependymal cyst in the fourth ventricle of a dog with successful surgical resection.

Is Antihelminthics Necessary in Postoperative Treatment of Intraventricular Neurocysticercosis? A Systematic Review.

OBJECTIVE: Patients with intraventricular neurocysticercosis (IVNCC) may require cerebrospinal fluid diversion surgery for late-onset hydrocephalus in the postsurgical period. Controversy exists regarding cysticidal treatment. Our main objective was to compare surgically treated cases of IVNCC that received postoperative anthelmintics with those that did not regard the incidence and treatment of late-onset hydrocephalus. METHODS: We searched the Medline database and extracted the following data: age, gender, stage of development of cysticercosis, type of operation, frequency of delayed hydrocephalus, cerebrospinal fluid diversion surgery, outcome, and follow-up. RESULTS: We analyzed 130 articles on intraventricular cysticercosis and identified 117 cases of isolated IVNCC and 314 patients in the case-control series who met inclusion criteria. There was no significant difference in postoperative delayed hydrocephalus between isolated IVNCC and case-control study groups. Children under the age of 16 received anthelmintic drugs more frequently during the postoperative period. Statistical relevance was observed in all patient groups regarding the application of steroids in favor of cysticidal therapy Endoscopy was a better option than craniotomy for cases of isolated IVNCC and case-control studies. Other variables were not relevant. CONCLUSIONS: Patients who received antihelminths did not show a statistically significant reduction in delayed hydrocephalus compared to individuals who did not receive after surgical resection of the parasite. Corticosteroid therapy prevailed in people who have been treated with anthelmintics. Children under the age of 16 were administered anthelmintic drugs more frequently during the postoperative period. Endoscopy was the preferred method for all groups, but some patients with cysts in the fourth ventricle required a craniotomy.

Treatment of brainstem and fourth ventricle lesions by the full neuroendoscopic telovelar approach.

OBJECTIVE: To explore the surgical techniques, advantages, and disadvantages of neuroendoscopic telovelar approach in the treatment of brainstem and fourth ventricle lesions. METHODS: The clinical data of 5 patients treated by neuroendoscopic telovelar approach from March 2020 to March 2022 were analyzed retrospectively. RESULTS: Among the 5 patients, there were 3 cavernous hemangiomas in pontine arm and 2 tumors in brainstem and fourth ventricle. All patients could successfully complete the operation, and 4 patients recovered well, other 1 patient discharged automatically for serious complications of other systems after the operation. CONCLUSION: The telovelar approach has gained popularity as a safe and effective strategy for lesions in fourth ventricular and brainstem. However, without removing the posterior arch of the atlas, it is difficult to enter the upper part of the fourth ventricle under a microscope. Transcranial neuroendoscopy can effectively compensate for the shortcomings of microscopy, whether used as an auxiliary measure for microsurgery or alone with proficient endoscopic techniques, it will provide greater application in minimally invasive surgery for fourth ventricle and brainstem lesions. By utilizing the excellent degree of freedom of transcranial neuroendoscopy, there is no need to open the posterior arch of the atlas, making the surgery more minimally invasive. However, the sample size of this study is small, and it was completed under the very mature neuroendoscopic technology of our team. Its general safety and practicality still require extensive clinical research validation.

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.

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.

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.

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.

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.