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.

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.

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.

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.

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.

Tumour-induced Osteomalacia Secondary to Intracranial Tumours - Report of 2 Cases.

Tumor induced osteomalacia (TIO) is a paraneoplastic syndrome which is mostly caused by a phosphaturic mesenchymal tumour mixed connective tissue variant (PMTMCT). These tumours do not have any specific site predilection but their presence in cranial compartment is very rare. Two cases of TIO secondary to phosphaturic mesenchymal tumour at the skull base are described ahead, one of which was in the posterior fossa and the other in middle cranial fossa. Early diagnosis and complete excision of PMT is essential in preventing morbidity secondary to osteomalacia. This case report stands distinct in highlighting a rare site of a phosphaturic mesenchymal tumour and the need to keep a high index of suspicion in cases of TIO especially wherein localization of the tumour is unsuccessful.

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.

Neuroendoscopy for post-infective hydrocephalus in children.

The treatment of hydrocephalus has changed in recent years with better imaging and introduction of endoscopic procedures as well as enhanced shunts. Indications of endoscopic third ventriculostomy (ETV) are now more refined with better quantification of outcome. This article reviews the current state of neuroendoscopy for infective hydrocephalus in children. The roles of third ventriculostomy as a primary procedure or after shunt malfunction, endoscopic interventions in multiloculated hydrocephalus and introduction of intraventricular lavage to salvage severely infected children are evaluated.

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.

Surgical Management of Rathke Cleft Cysts.

BACKGROUND: The diagnosis of Rathke cleft cysts (RCC) has increased in recent times as a result of improvements in imaging techniques; however, symptomatic patients are uncommon and accurate preoperative diagnosis may sometimes be difficult. The indications of surgical management protocol are evolving. We aim to provide a comprehensive review of clinical, imaging, and histopathologic features with operative management strategies along with outcome and prognosis in RCC. METHODS: A retrospective analysis (2003-2015) was performed of 58 consecutive cases of RCC seen in a surgical unit. Twenty-seven surgically treated symptomatic RCCs were further evaluated for their clinical presentation, imaging characteristics, surgical approaches, and intraoperative findings. RESULTS: Headache was the most common presenting complaint followed by visual deficit. Hormonal abnormality was observed in 13 patients. On magnetic resonance imaging, the characteristic intracystic nodule was identified in 6 patients. Transsphenoidal surgery for cyst excision was performed in all 27 patients with an endoscopic route in 25 patients and radical excision was performed in 17 patients. The pituitary stalk and the normal gland were preserved in all patients. Headache improved in 96% of patients and visual field defect resolved in all. Around 46% had improvement of the anterior pituitary axis. New permanent hormone deficiency was not observed. The recurrence rate was 3.7% after a minimum of 18 months follow-up. CONCLUSIONS: RCCs are an uncommon disease with a wide spectrum of clinical and radiologic features. Endonasal endoscopic transsphenoidal surgery provides excellent clinical and endocrinologic improvement. We believe that radical excision does not necessarily result in endocrinologic impairment and may have a better impact on recurrence and cyst resolution.

Primary intraventricular meningiomas in children-experience of two cases with review of literature.

Meningiomas are rare in the pediatric age group, more so in the intraventricular location. They arise in the lateral ventricles from the arachnoid cells contained within the choroid plexus, in the third ventricle from the velum interpositum and in the fourth ventricle from the choroids. These tumors are usually large and have an aggressive behaviour. Surgical management of intra-ventricular meningiomas is challenging because of their deep location, large size at presentation and increased vascularity. The authors report two such cases who presented with symptoms of raised intra cranial pressure and on evaluation were found to have associated hydrocephalus. Both these patients underwent surgical excision of the tumour by frontal transcortical approach and histopathology report confirmed transitional meningioma in them. Only twenty seven cases of intraventricular meningiomas in children have been reported till date. Their definitive treatment is surgery alone and total excision of the tumor is curative. Possibility of neurofibromatosis as a differential should also be considered in their management.

Endoscopic Third Ventriculostomy: Success and Failure.

Endoscopic third ventriculostomy (ETV) has now become an accepted mode of hydrocephalus treatment in children. Varying degrees of success for the procedure have been reported depending on the type and etiology of hydrocephalus, age of the patient and certain technical parameters. Review of these factors for predictability of success, complications and validation of success score is presented.