Intraventricular Tumors: Surgical Considerations in Lateral and Third Ventricular Tumors.

Management of lateral and third ventricular tumors has been a challenge for neurosurgeons. Advances in imaging and pathology have helped in a better understanding of the treatment options. Technical refinement of microsurgical technique and addition of endoscopy has enabled more radical excision of tumors, when indicated, and added more safety.A proper understanding of the pathology at various ages and treatment options is continuously evolving. Many pediatric tumors are amenable to conservative surgical methods with effective complementary treatments. However, radical surgery is required in many adults as the main treatment and for many benign tumors. Various intraventricular lesions encountered and their surgical management is reviewed here for their efficacy, safety, and outcome, encompassing changes in our practice over the last 20 years.

Cystic Cerebral Cavernous Malformations: Report of Five Cases and a Review of Literature.

Introduction Cerebral cavernous malformations (CCMs) account for about 5 to 13% of intracranial vascular malformations. Cystic cerebral cavernous malformations (cCCMs) are a rare morphological variant and can cause diagnostic and therapeutic dilemmas. We describe our five such cases and review the existing literature on this entity. Methods A search of the PubMed database for cCCMs was done, and all articles in English emphasizing the reporting of cCCMs were selected. A total of 42 publications describing 52 cases of cCCMs were selected for analysis. Epidemiological data, clinical presentation, imaging features, the extent of resection, and outcome were analyzed. Radiation-induced cCCMs were excluded. We have also described five of our cases of cCCMs and reported our experience. Results The median age at presentation was 29.5 years. Twenty-nine patients had supratentorial lesions, 21 had infratentorial lesions, and 2 had lesions in both compartments. Among our four patients, three had infratentorial lesions, whereas one had a supratentorial lesion. Multiple lesions were seen in four patients. A majority (39) had symptoms of mass effect (75%), and 34 (65.38%) had raised intracranial pressure (ICP), whereas only 11 (21.15%) had seizures. Among our four operated patients, all of them had symptoms of mass effect, and two of them also had features of raised ICP. The extent of resection was gross total in 36 (69.23%), subtotal in 2 (3.85%), and not reported in 14 (26.93%). All four of our operated patients underwent gross total resection, but two of them underwent a second surgery. Of the 48 patients in whom the surgical outcome was reported, 38 improved (73.08%). One showed a transient worsening followed by improvement, one developed a worsening of the pre-existing focal neurological deficit (FND), two developed a new FND, and 5 had no improvement in their FNDs. Death occurred in one patient. All four of our operated patients improved after surgery, although three of them showed a transient worsening of FNDs. One patient is under observation. Conclusion cCCMs are rare morphological variants and can cause considerable diagnostic and therapeutic dilemmas. They should be considered in the differential diagnosis of any atypical cystic intracranial mass lesion. Complete excision is curative, and the outcome is generally favorable; although transient deficits may be seen.

Neuroendoscopy in the management of pineal region tumours in children.

INTRODUCTION: Pineal region tumours (PRTs) are more common in children and represent a wide variety of lesions. The practise of a radiation test dose is obsolete and a biochemical/histological diagnosis is recommended before further therapy. Many patients present with hydrocephalus. Advances in neuroendoscopic techniques have allowed safe and effective management of this obstructive hydrocephalus with an opportunity to sample cerebrospinal fluid (CSF) and obtain tissue for histopathology. Definitive surgery is required in less than a third. Endoscopic visualisation and assistance is increasingly used for radical resection, where indicated. METHODOLOGY: Our experience of endoscopic surgery for paediatric PRTs from 2002 to 2021 is presented. All patients underwent MRI with contrast. Serum tumour markers were checked. If negative, endoscopic biopsy and endoscopic third ventriculostomy (ETV) were performed; and CSF collected for tumour markers and abnormal cells. For radical surgery, endoscope-assisted microsurgery procedures were performed to minimise retraction, visualise the extent of resection and confirm haemostasis. RESULTS: M:F ratio was 2:1. The median age of presentation was 11 years. Raised ICP (88.88%) was the commonest mode of presentation. Nineteen patients had pineal tumours, one had a suprasellar and pineal tumour, one had disseminated disease, while six had tectal tumours. The ETB diagnosis rate was 95.45%, accuracy rate was 83.3% and ETV success rate was 86.96%. CONCLUSION: Neuroendoscopy has revolutionised the management of paediatric PRTs. It is a safe and effective procedure with good diagnostic yield and allows successful concurrent CSF diversion, thereby avoiding major surgeries and shunt implantation. It is also helpful in radical resection of lesions, where indicated.

Intraventricular Craniopharyngiomas-Overcoming Their Relative Inaccessibility: Institutional Experience With a Review of Literature.

Introduction: Craniopharyngiomas constitute 2-4% of intracranial neoplasms. Intraventricular craniopharyngiomas (IVCrs) are the rarely encountered varieties of these lesions. Objective: The objective of the study was to study the special features in clinical presentation, imaging, management, and surgical outcome of IVCrs. Materials and Methods: This retrospective analysis included the combined experience from two tertiary care institutions. Medical records of histopathologically proven cases of IVCrs from January 1994 to June 2021 were assessed, and images were analyzed based on the criteria by Migliore et al. for inclusion of solely intraventricular lesion with the third ventricular ependyma demarcating it from the suprasellar cistern. Results: Among the 25 patients included (mean age: 35.4 years), the most common presentation included headache (n = 21, 84%), vomiting and other features of raised ICP (n = 18, 72%), visual complaints (n = 12, 48%), and endocrinopathies (n = 11, 44%). Fifteen had predominantly cystic tumors, two were purely solid, and eight were of mixed consistency. Primary open microsurgical procedures were performed in 18 (72%) patients, of which four (16%) were endoscope-assisted. Seven (28%) underwent a purely endoscopic procedure. One underwent a staged surgery with endoscopic cyst fenestration and intracystic interferon (IFN)-alpha therapy, followed by microsurgical excision. Complete excision was achieved in 10 patients, near-total in nine, and partial excision in six. Four patients underwent a ventriculoperitoneal shunt (one before the definitive procedure). At a median follow-up of 36 months (range:11-147 months), five patients developed a recurrence, and one had a stable small residue. This patient and two others with small cystic recurrences were observed. One patient was managed with radiotherapy alone. Another underwent re-surgery after a trial of radiotherapy, and the last patient developed a local recurrence, which was managed with radiotherapy; he then later developed an intraparenchymal recurrence, which was operated. Conclusion: Purely IVCrs present with raised intracranial pressure, and visual disturbances are less common. Their deep-seated location and limited surgical field-of-view makes minimally invasive endoscopic-assisted surgery most suitable for their excision. The thin-walled cystic lesions may be occasionally adherent to the ependymal wall in close vicinity to the thalamus-hypothalamus complex, making complete excision difficult. Their responsiveness to radiotherapy, often leads to a gratifying long-term outcome.

Paediatric giant cavernomas: report of three cases with a review of the literature.

INTRODUCTION: Cavernous angiomas of the brain (CCM) are being increasingly diagnosed, especially in the paediatric age group. Though classic presentations with haemorrhage or seizures are well recognised, presentation as a large lesion with mass effect is rare and creates difficulty in diagnosis as well as management. METHODS: Our cases of paediatric giant CCMs that presented as a 'mass lesion' are reported here, and the PubMed database for giant CCMs in the paediatric population is reviewed. All articles where the size of the lesion was reported to be > 4 cm were selected for analysis to study the varying modes of presentation, treatment, and outcome; to gain a proper perspective on this distinct entity of 'giant CCMs'. RESULTS: Analysis of a total of 53 cases (inclusive of our 3 cases) reported so far showed slight male preponderance (58.49%). The largest reported lesion was 14 cm in largest diameter. Most of the lesions (83.02%) occurred in the supratentorial region. In the infratentorial region, paediatric giant CCMs were more commonly seen in the cerebellum than in the brainstem. Seizures were observed in 47.17% at presentation. Features of mass effect were the mode of presentation in all our cases, and literature analysis has shown raised intracranial pressure in 37.74% (20 patients) and focal neurological deficit in 33.96% (18 patients) at presentation. Macrocephaly was seen in younger children up to the age of 7 years (16.98% or 9 patients). Gross total resection was carried out (with a good outcome) in all our cases and in 36 of the other 49 analysed patients who were operated on. DISCUSSION: About one-fourth of CCMs occur in paediatric patients. Giant CCMs are rare but can present in children even in the immediate post-natal period. Features of a mass lesion such as raised intracranial pressure, macrocephaly, and focal neurological deficit are much more common than their smaller counterparts. Their appearance on imaging also often causes diagnostic dilemmas with other intracranial mass lesions. Timely surgery with standard microsurgical principles leads to a favourable outcome in the majority. CONCLUSION: Giant CCMs, though rare, often present as a diagnostic challenge. Presentation with mass effect is common, and complete microsurgical excision remains the mainstay of treatment. Though transient neurological deficits may be encountered with this strategy, the long-term outcome remains favourable.