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.

Encouraging Experience with Image-Guided Pencil Beam Scanning Proton Therapy in Craniopharyngioma-First Case Series From India.

OBJECTIVE: We report our early clinical experience with image-guided, pencil beam scanning proton beam therapy (PBS-PBT) for residual and recurrent craniopharyngioma. METHODS: Between September 2019 and January 2023, 19 consecutive patients with residual or recurrent craniopharyngioma, suitable for radiotherapy and treated with image-guided PBS-PBT were analyzed. We documented detailed dosimetric data, acute toxicities, early outcomes, and imaging response on follow-up magnetic resonance imaging scans. RESULTS: A total of 19 patients (11 males and 8 females) with residual or recurrent craniopharyngioma were treated during the study period. The median age of the cohort was 14 years (range, 3-33 years). The histology of most lesions was the adamantinomatous subtype (95%). The most common clinical presentation (before PBT) and most common endocrine deficit was visual disturbance (79%) and hypocortisolism (74%), respectively. Of the 19 patients, 13 had recurrent craniopharyngioma, and 5 had undergone radiotherapy previously. Five patients (26%) had undergone surgery ≥3 times before proton therapy. The median dose delivered was 54 GyE. The most common acute toxicity was grade 1 alopecia (63%). No patient experienced grade ≥3 acute toxicity. With a median follow-up of 18 months (range, 3-40 months), 12 patients showed shrinkage of the residual tumor and/or cyst, and 4 showed a dramatic cyst reduction at 3-9 months of follow-up. Two patients experienced a reduction in both solid and cystic components, with the remaining experiencing a reduction in the cystic component only. The remaining 8 patients had stable disease on magnetic resonance imaging, with 100% disease control and overall survival. Visual function remained stable after treatment. CONCLUSIONS: Our preliminary experience with modern PBS-PBT and image guidance for craniopharyngioma is encouraging. Proton therapy in our cohort was well tolerated, resulting in limited toxicity and promising early outcomes.

Neuroendoscopy: intraventricular and skull base tumor resection in children.

During the last 30 years, the neurosurgeons have witnessed a revolution in the practice of interventricular surgery. The advent of neuroendoscopy at the end of the 1980s has allowed a minimally invasive management of a very large series of pathologies in pediatric neurosurgery ranging from hydrocephalus to arachnoid cyst to intraventricular tumors. The progresses in the management of hydrocephalus, intracranial cyst, and the fluid filled collection nevertheless has been more rapid and radical due to the simpler equipment that is necessary to perform this kind of surgery. The intraventricular tumors instead have been addressed in a slower way, and for many years, the only endoscopic procedure that was allowed on interventricular tumors was a biopsy associated with the management of hydrocephalus. Only very small tumors have been considered operable for complete removal during many years due to the limitations of the neuroendoscopic equipment and to the small calibers of the working channel. More recently, the advent of new devices and new surgical techniques are offering new perspectives on the possibility of intraventricular tumor surgery in children. In this review, we describe the historical perspective of the learning curve of intraventricular tumor surgery under neuroendoscopic control and try to offer a view of the future perspective in the removal of larger intraventricular tumors, analyzing the main indications for intraventricular endoscopic tumor surgery. We offer as well an historical perspective of the evolution of skull base surgery and endonasal transsphenoidal approach for skull-based tumors in children. This kind of surgery that has acquired widespread acceptance for many pathologies in adult age has diffused more slowly in pediatric neurosurgery due to the anatomical limitation observed in these age range. Also in this field, the slow evolution of the technique and of the technology available to neurosurgeons has allowed a very significant expansion of indication for the minimally invasive removal of skull base tumors in children.

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.

Study of Neurodevelopmental Outcome in Patients with Non-tumoral Hydrocephalus with Shunt Surgery Done in Infancy.

BACKGROUND: Hydrocephalus (HC) is a common neurological disorder presenting in infancy, with a myriad of etiologies requiring early neurosurgical intervention. OBJECTIVE: To study neurodevelopmental outcome in patients with HC with shunt surgery done in infancy. MATERIALS AND METHODS: This was an observational retrospective cohort study of 50 pediatric patients (2 years to 16 years of age). These patients were diagnosed with HC and were operated on with ventriculo-peritoneal shunt (VP shunt) insertion in infancy (did not include patients with brain tumors) and then later following in the neurology outpatient department (OPD). Clinical records and neurodevelopmental assessment (intelligence quotient [IQ]/development quotient [DQ] and vision and hearing assessment) were reviewed. RESULTS: Only 50% of the patients with congenital HC were diagnosed at birth, which included patients who had been diagnosed antenatally and they had lesser complications and better intellectual outcome (P = 0.12), compared with those who presented later with HC. Patient-related factors such as etiology of HC, antenatal diagnosis, and requirement of shunt revisions had poor correlation with neurodevelopmental outcome. Patients with late postoperative complications had significantly poor neurodevelopmental outcome (P ≤ 0.001). Patients with post-meningitis HC required a significantly higher number of shunt revisions than patients with other causes (P = 0.04). CONCLUSION: Better neurodevelopmental outcome depends on early diagnosis and early referral for the management than the cause of HC. Regular head circumference monitoring is the most feasible and sensitive screening tool for early pickup. Larger studies are needed for accurate prognostication.

Transnasal Endoscopic Surgery for Suprasellar Meningiomas.

AIM: Endoscopic trans-nasal surgery has evolved a long way from the days of narrow corridors with high rates of cerebrospinal fluid (CSF) leak to the present state of HD optics with better tissue differentiation, extended approaches, and use of vascularized flaps for defect closure. Trans-nasal approach is an established technique for pituitary tumors practiced worldwide. However, trans-nasal endoscopic excision of suprasellar meningiomas provides a tougher challenge in terms of instrument manipulation, tumor excision with good visual outcome, and a robust defect closure to prevent CSF leaks. MATERIALS AND METHODS: Out of 83 cases of midline anterior cranial fossa meningiomas operated over 14 years, our experience in 12 cases of suprasellar meningiomas for radical resection via the trans-nasal endoscopic route is discussed. RESULTS: Amongst these, six were excised via primary extended endoscopic trans-sphenoidal surgery, four cases had a residual lesion or recurrence after primary transcranial surgery, and two cases involved a combined transcranial and extended endoscopic approach. Visual improvement along with resolution of headache was seen in all patients postoperatively. None of the patients had CSF leak requiring further repair. Syndrome of inappropriate antidiuretic hormone was found in one patient, which was transient and easily corrected. CONCLUSION: Trans-nasal endoscopic surgery for suprasellar meningiomas is an effective technique that provides results of tumor excision comparable to the transcranial approach in suitable cases. Visual outcome was found to be superior, and rates of CSF leak were remarkably reduced with vascularized flap. However, each case must be assessed individually and lateral extension beyond the optic canals with internal carotid artery encasement must be considered before planning surgery.

A comparison of Adult and Pediatric Hydrocephalus.

Hydrocephalus is a common clinical problem encountered in neurosurgical practice. With greater subspecialisation, pediatric neurosurgery has emerged as a special discipline in several countries. However, in the developing world, which inhabits a large pediatric population, a limited number of neurosurgeons manage all types of hydrocephalus across all ages. There are some essential differences in pediatric and adult hydrocephalus. The spectrum of hydrocephalus of dysgenetic origin in a neonate and that of normal pressure hydrocephalus of the old age has a completely different strategy of management. Endoscopic third ventriculostomy outcomes are known to be closely associated with age at presentation and surgery. Efficacy of alternative pathways of CSF absorption also differs according to age. Managing this disease in various age groups is challenging because of these differences in etiopathology, tempo of the disease, modalities of investigations and various treatment protocols as well as prognosis.

Neuroendoscopy in the Surgical Management of Lateral and Third Ventricular Tumors: Looking Beyond Microneurosurgery.

BACKGROUND: Intraventricular tumors pose a surgical challenge because of the difficulty in reaching their deep location through safe corridors and their adherence or proximity to vital neurovascular structures. Although microneurosurgery is the mainstay of surgical management, neuroendoscopy aided by adjuncts, namely, navigation and ultrasonic aspirators, has made a great contribution to improving surgical results. OBJECTIVE: This article reviews the experience of a neurosurgical unit with endoscopic procedures for intraventricular tumors. The current indications, benefits, and complications of neuroendoscopy are described. MATERIALS AND METHODS: This is a retrospective, observational study of lateral and third ventricular tumors tackled either purely with an endoscope or with its assistance over 19 years in a single unit at Bombay Hospital Institute of Medical Sciences, Mumbai. RESULTS: Of a total of 247 operated patients with intraventricular tumors, 85 cases operated using an endoscope were included. The majority of the patients had a tumor in the third ventricle (n = 62), whereas 23 patients had tumor in the lateral ventricle. The most common pathologies were colloid cyst and arachnoid cyst (n = 18). An endoscope was used for microsurgical assisted excision of tumors in 31 cases, biopsy in 24, cyst fenestration in 23, and pure endoscopic excision in seven cases. CONCLUSION: Microsurgery remains the gold standard for the removal of giant, vascular intraventricular tumors. However, endoscopic fenestration or excision of cysts and biopsy have become better alternatives in many cases. Endoscope-assisted microsurgery affords safety and helps in achieving a more complete excision.

Giant extradural spinal schwannoma in a non-neurofibromatosis child-case report and review of literature.

Spinal schwannoma is a rare occurrence in non-neurofibromatosis (NF) pediatric patients, especially in the extradural space extending beyond two vertebral levels. Within this age group, the common extradural tumors are either soft tissue sarcomas or metastasis, often with vertebral bony involvement. Spinal schwannomas are usually benign, slowly progressive, well-defined, intradural extramedullary lesion showing homogenous contrast enhancement on imaging. Though its clinical presentation may be with marked neurological involvement, timely surgical excision usually leads to a quick recovery of the deficits in the young age.This case report describes a giant, spinal, benign schwannoma in a 6-year-old boy which was extradural in location. The lesion was resected completely, and since then, he has been asymptomatic, tumor-free for over 3 years.

Experience with Management of Intracranial Arachnoid Cysts.

OBJECTIVES: The aim of this study was to analyze the demographics, clinical presentation, and surgical outcome of intracranial arachnoid cysts and to review the surgical options. METHODS: This is a retrospective observational study of 56 cases of symptomatic arachnoid cysts among all age groups treated between 2004 and 2020 at the Bombay Hospital, Mumbai. Endoscopic fenestration, microsurgical cyst excision, and shunt insertion were the interventions performed. Clinical presentation, cyst reduction post-intervention, and complications were studied. The follow-up period varied from 1 month to 16 years. Statistical analysis was done for 43 patients with a minimum of 3 years' follow-up. RESULTS: In all, 75% of patients were <18 years of age. Of these, the majority were between the age of 1 and 10 years. There were 14 cases of temporal, 13 cases of retrocerebellar, 10 cases of quadrigeminal cistern, and 7 cases each of interhemispheric and suprasellar arachnoid cysts. The most common clinical presentation was headache and vomiting. Concomitant hydrocephalus was seen on imaging in 24 cases. Endoscopic fenestration of cyst was the most routinely performed procedure (35 cases). Four patients of endoscopic fenestration underwent a redo endoscopic procedure on follow-up. Post-operative reduction in cyst size was found to be significantly better after endoscopic fenestration (p < 0.05). CONCLUSION: Though the best available treatment for arachnoid cysts remains controversial, surgery has been found to be beneficial in symptomatic cases. Endoscopic fenestration is considered the first-line surgical option and it may be followed by shunt, if necessary. Shunts may be preferred in very young children where there is associated hydrocephalus/macrocephaly.

Microsurgery and Neuromodulation for Facial Spasms.

Facial spasms are of various types. Hemifacial spasm (HFS) is characterized by unilateral tonic-clonic contractions of facial muscles, following a specific pattern of disease progression. It has well-delineated clinical, radiological and electrophysiological features. We have conducted an extensive review of existing literature on the subject, as regards etiopathogenesis, clinical features, investigations and management options for facial spasms. Primary Hemifacial spasm (HFS) may be treated using pharmacotherapy, botulinum toxin injections or microvascular decompression surgery. Microvascular decompression has the potential to reverse the pathological changes of the disease and has proved to be the most successful of all treatment options. Other facial spasms are exceedingly difficult to treat and may need neuromodulation as an option. The following article attempts to review the clinical features and therapeutic approaches to managing patients with facial spasms.

Scedosporium Apiospermum: Rare Cause of Brain Abscess in an Immunocompetent Patient.

Scedosporium apiospermum is a filamentous fungus causing a broad spectrum of clinical diseases especially in those who are immunocompromised. The common sites involved are lungs, skin, sinuses, eyes, bones, joints, and central nervous system (CNS). CNS is involved in invasive Scedosporiosis in the form of a cerebral abscess. An antecedent event of either near-drowning or history of some trauma is present in the majority of the cases where the patients' immune response remains intact. Prognosis is generally poor since the majority of the patients have coexistent medical morbidity. Surgical drainage followed by adjuvant antifungal, i.e., voriconazole therapy offers the best possible chance for survival in these patients. This case report discusses a rare event of brain abscess caused by S. apiospermum in an immunocompetent patient without any preceding precipitating factor.

Evolution of Pituitary Surgery.

Pituitary tumors may well be the most common brain tumors with a mean incidence of 16.7%. Even small tumors become symptomatic when they arise from functioning cells and produce devastating effects on the body. The nonfunctioning tumors may become quite large before producing symptoms due to raised intracranial pressure or mass effect on the surrounding structures, most commonly, the optic apparatus. Many of them remain asymptomatic through life. Evolution of pituitary surgery is testimony to the advances in diagnostic and surgical techniques in neurosurgery and improved understanding of 360° of surgical skullbase anatomy as well as the need to provide not only immediate good postoperative results but also a long-lasting relief. Despite considerable advances in medical treatment as well as focussed radiation techniques, surgery remains the primary treatment in many of these tumors. Visual improvement, hormonal cure, avoidance of hypopituitarism, and neurological deficit remain immediate goals of surgery. Long-term cure or remission may require a multidisciplinary approach.

Sellar Pathologies Mimicking Pituitary Tumors.

The most common sellar pathology that merits neurosurgical attention is the pituitary adenoma. However, some developmental, inflammatory, and neoplastic lesions may primarily or secondarily involve the sella, mimicking pituitary tumors. Advances in imaging and endocrinological assessment have helped in the recognition of these less common sellar, supra sellar pathologies, which may occasionally create confusion in management. The most common developmental anomaly is the Rathke's cleft cyst and an increasingly recognized inflammatory pathology is the spectrum of hypophysitis. Neoplasms, viz. Craniopharyngioma, Germinoma, Langerhans's cell histiocytosis or metastasis, have more distinctive features in various age groups and are more likely to be correctly diagnosed on current imaging and managed accordingly. This review looks at mainly intraparenchymal pathologies, namely Rathke's Cleft Cyst and various hypophysitides, and will discuss their management strategies.

India's First Proton Beam Therapy Pediatric Patient.

We recently started India's first proton beam therapy facility. Proton beam therapy because of its unique physical characteristics of minimal exit dose has an unequivocal dosimetric superiority over high-end photon/standard X-ray beam therapy and is particularly advantageous in growing children with curable cancers in view of their very high probability of long-term cures. We hereby report a case of a 7-year-old boy with a craniopharyngioma which had been subtotally resected and was subsequently treated with modern pencil beam proton therapy under high-precision image guidance. This is the first ever child ever to be treated with proton therapy in India.