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.

Cervicomedullary glioblastoma: A report of two cases with review of literature.

Background: Cervicomedullary glioblastoma is an extremely rare clinical entity and the principles of its management are not well understood. Case Description: We report two cases of cervicomedullary glioblastoma in young patients aged 12 and 30 years with contrasting clinical presentation and outcomes. The 12-year-old child had rapid onset bulbar symptoms, with frank infiltration of the medulla due to which the patient succumbed within 4 weeks of surgery. The 30-year-old adult had a relatively slow disease onset and progression and made a good neurological recovery without disease progression at 16 months after surgery. To the best of our knowledge, we also report only the second adult patient in the literature with a dorsally exophytic cervicomedullary glioblastoma. Difficulties in diagnosis and management are discussed with a review of the pertinent literature. Conclusion: The overall outcome depends on the rapid progression and severity of preoperative symptoms and the degree of tumor infiltration noted in imaging and during surgery.

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.

Spinal Nerve Root Extradural Melanocytoma Progressing to Malignant Melanoma: A Case Report with Review of Literature.

Melanocytomas are rare benign pigmented tumors arising from the leptomeninges with a very remote chance of progressing to malignant melanoma. They have a predilection for occurring in the posterior fossa or in the intradural extramedullary region of the cervical spine. We report the first case of malignant transformation of a nerve root (extradural) melanocytoma wherein immunotherapy has been added for its treatment. Only four such cases of malignant transformation of central nervous system melanocytoma have been reported in the literature. Definite diagnosis in such cases is based on immunohistochemistry evaluation. Surgical resection with adjuvant radiotherapy and immunotherapy is the recommended treatment.

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.

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.

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.

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.

Primary Suprasellar Hypothalamic CNS Lymphoma in an Immunocompetent Adult: A Case Report and Review of Literature.

BACKGROUND: Primary suprasellar central nervous system lymphoma (PCNSL) of the hypothalamus is a rare entity. MATERIAL AND METHODS: We report a case of a 49-year-old, healthy male presented with features of diabetes insipidus. Imaging features showed a mass in the suprasellar region involving the hypothalamus mainly tuber cinereum and infundibulum. RESULTS: Preoperative fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) showed increased uptake in the mass. Biopsy revealed a diffuse type-B PCNSL. The present case emphasizes the importance of considering the diagnosis of hypothalamic lymphoma and the utility of FDG PET-CT in such situations. CONCLUSION: To our knowledge, only eight cases of suprasellar hypothalamic PCNSL have been reported in the literature.

Role of Secondary Endoscopic Third Ventriculostomy in Children: Review of an Institutional Experience.

BACKGROUND: Endoscopic third ventriculostomy (ETV) has become a standard and safe procedure for obstructive hydrocephalus. ETV can also play an important role in children presenting with shunt malfunction with an added advantage of shunt independence. Secondary ETV can be defined as either a redo endoscopic ventriculostomy done after primary ETV stoma closure or that done in cases presenting with shunt malfunction. OBJECTIVE: The aim of our study was to evaluate the role of secondary ETV in the pediatric age group patients. METHODS: This is a retrospective analysis of 36 children (<18 years) who underwent ETV after shunt malfunction and 4 children with ETV done after previous ETV stoma closure from 2004 until 2018. In all patients, the obstructive pattern suggesting aqueduct outflow obstruction was observed on MRI. Patients were followed up for a mean period of 4.25 years. RESULTS: ETV was considered successful if the patient avoided a shunt insertion later on in their life. Considering this definition, a success rate of 72% was observed with secondary ETV for shunt malfunction whereas a success rate of 75% was observed after primary ETV failure without any major side effects in any of the patients. CONCLUSION: ETV can be considered a primary treatment modality in children with shunt malfunction and has a good success rate in cases presenting with closure of previously performed ETV stoma.

Endonasal endoscopic skullbase surgery in children.

OBJECTIVE: The role of endonasal endoscopic approach for pathologies in the paediatric population is evolving and has still not been accepted as standard of care in neurosurgery. It represents a challenge in terms of narrow access, instrument manipulation and adequate reconstruction of defects. We have described our experience in 49 cases from a single neurosurgical unit in paediatric skull base surgeries through this approach over the last 12 years. MATERIAL AND METHODS: A case series of 59 paediatric skull base surgeries in 49 children through endoscopic endonasal route over the last 12 years is presented. The age ranges from 4 months to 18 years. Out of 49 cases, 22 cases were of craniopharyngiomas, 8 cases of pituitary adenomas, 5 cases with CSF rhinorrhea, 5 cases with meningoencephalocele, 3 cases of Rathke's cleft cysts, 2 cases of odontoidectomy and 4 miscellaneous cases viz. mucocele, hypothalamic glioma, esthesioneuroblastoma and epidermoid. CSF leaks were repaired with free graft in the initial years and by vascularized flap more recently. RESULTS: The goal of surgery was achieved in all but two cases in whom the tumour excision was unsatisfactory due to failure of the cyst wall to collapse after decompression. Extent of tumour excision was not compromised by the choice of this approach. Revision surgery for CSF leak was required in three patients. Local vascularized nasoseptal flap has been possible even in very young patients and has now become the standard for reconstruction. CONCLUSION: In spite of the challenges posed by small nostrils and ill-developed sinuses in the paediatric age group, surgery from endoscopic endonasal corridor is possible to be carried out successfully in selected cases.

Use of animal models in peripheral nerve surgery and research.

Animal models are widely used in research of peripheral nerve injury and regeneration, since there are only minor differences in the anatomy of peripheral nerves and the physiology of nerve regeneration between the humans and animals. Animal models, especially rodents, are widely used for this purpose. This narrative review provides a brief overview of the role of animal models in peripheral nerve surgery and research.

Pediatric spinal infections-a review of non-tuberculous infections.

Pediatric spinal infection includes spinal epidural abscess, spondylodiscitis and intradural (extramedullary and intramedullary) spinal infections. However, these entities are extremely rare and no clear guidelines exist for their management. Pertinent literature was searched and a detailed narrative review of this topic is presented.

Combined endoscopic approach in the management of suprasellar craniopharyngioma.

INTRODUCTION: Craniopharyngiomas are dysontogenic tumors with benign histology but aggressive behavior. The surgical challenges posed by the tumor are well recognized. Neuroendoscopy has recently contributed to its surgical management. This study focuses on our experience in managing craniopharyngiomas in recent years, highlighting the role of combined endoscopic trans-ventricular and endonasal approach. CASE SERIES: Ninety-two patients have been treated for craniopharyngioma from 2000 to 2016 by the senior author. A total of 125 procedures, microsurgical (58) and endoscopic (67), were undertaken. Combined endoscopic approach was carried out in 18 of these patients, 16 children and 2 young adults. All of these patients presented with a large cystic suprasellar mass associated with hydrocephalus. In the first instance, they were treated with a transventricular endoscopic procedure to decompress the cystic component. This was followed by an endonasal transsphenoidal procedure for excision within the next 2 to 6 days. All these patients improved after the initial cyst decompression with relief of hydrocephalus while awaiting remaining tumor removal in a more elective setting. Gross total resection could be done in 84% of these patients. Diabetes insipidus was the most common postsurgical complication seen in 61% patients in the immediate period but was persistent in only two patients at 1-year follow-up. None of the children in this group developed morbid obesity. There was one case of CSF leak requiring repair after initial surgery. Peri-operative mortality was seen in one patient secondary to ventriculitis. DISCUSSION: The patients who benefit most from the combined approach are those who present with raised intracranial pressure secondary to a large tumor with cyst causing hydrocephalus. Intraventricular endoscopic cyst drainage allows resolution of hydrocephalus with restoration of normal intracranial pressure, gives time for proper preoperative work up, and has reduced incidence of CSF leak after transnasal surgery. CONCLUSION: Combined endoscopic approach thus gives a unique opportunity to remove these lesions more radically with less morbidity.

Upper Cervical Bronchogenic Cyst: A Rare Condition at a Rare Location.

Intraspinal bronchogenic cyst (SBC) is a rare but important cause of spinal cord compression, commonly seen in the cervicothoracic spine. We report a case of a 43-year-old male, presenting with complaints of neck pain, radiating to right shoulder, with numbness of right hand and fingers. Magnetic resonance imaging of the cervical spine revealed an intradural extramedullary, ventral cystic lesion extending from C2 to C4 vertebral levels. Complete surgical excision was performed, and the patient had a complete relief of symptoms postoperatively. Only 11 cases of SBCs have been reported in literature. We discuss the peculiar location of this lesion, possible embryological reasons and the overall surgical outcome of SBC.

Posterior column reconstruction improves fusion rates at the level of osteotomy in three-column posterior-based osteotomies.

PURPOSE: To determine the incidence of pseudarthrosis at the osteotomy site after three-column spinal osteotomies (3-COs) with posterior column reconstruction. METHODS: 82 consecutive adult 3-COs (66 patients) with a minimum of 2-year follow-up were retrospectively reviewed. All cases underwent posterior 3-COs with two-rod constructs. The inferior facets of the proximal level were reduced to the superior facets of the distal level. If that was not possible, a structural piece of bone graft either from the local resection or a local rib was slotted in the posterior column defect to re-establish continual structural posterior bone across the lateral margins of the resection. No interbody cages were used at the level of the osteotomy. RESULTS: There were 34 thoracic osteotomies, 47 lumbar osteotomies and one sacral osteotomy with a mean follow-up of 52 (24-126) months. All cases underwent posterior column reconstructions described above and the addition of interbody support or additional posterior rods was not performed for fusion at the osteotomy level. Among them, 29 patients underwent one or more revision surgeries. There were three definite cases of pseudarthrosis at the osteotomy site (4%). Six revisions were also performed for pseudarthrosis at other levels. CONCLUSION: Restoration of the structural integrity of the posterior column in three-column posterior-based osteotomies was associated with > 95% fusion rate at the level of the osteotomy. Pseudarthrosis at other levels was the second most common reason for revision following adjacent segment disease in the long-term follow-up.