Natural history of post-operative non-functioning pituitary adenomas - a single centre cohort analysis.

PURPOSE: To study behaviour of endonasally operated non-functioning pituitary adenomas (NFPA) and propose a cost-effective stratified follow-up regimen. METHODS: A single centre retrospective cohort analysis from June 2009 till December 2019. All endonasally operated pituitary adenomas were identified with sub-analysis of the NFPA's. Patients of all age groups with radiological follow-up more than 30 months were included. Patients with any kind of cranial intervention performed < within 30 months of surgery were excluded. The post-operative MRI for this cohort was evaluated until either any intervention was performed or until the last follow-up. The maximal tumour diameter in any plane (mm) was measured from the MRI scans. The annual growth rate and the statistical relationship between age, sex, IHC, Ki-67, resection %, residual tumour was calculated. RESULTS: Out of 610 pituitary adenomas identified in the dataset, 116 patients met the inclusion criteria. Follow-up period ranged from 30 to 142 months (mean 78.5 months). A strong relationship existed between predicting tumour progression with first post-operative residue size (p = .001). A statistically significant relationship was found to be present between tumour growth and a residue of less than 10 mm diameter and 11-20 mm in diameter (Log rank p value .0216). On average, each patient with a residue < 5mm had MRI scans costing 976 £. CONCLUSION: Based on statistical analysis and internal validation of the growth rate of the residue, we have proposed MRI follow-up scans. These recommendations have the potential to save more than 300 £per patient towards MRI costs and can lay down a marker for defining time interval of serial scans for post-operative NFPA's.

Intracranial Myxoid Mesenchymal Tumour with EWSR1-ATF1 Fusion Sans Myxoid Stroma - Report of A Newer Entity with Brief Review of Literature.

Intracranial myxoid mesenchymal tumors (IMMTs) are a relatively new group of tumors, first described in 2017. We report this rare variant in a 27-year-old female which was initially suspected to be a high-grade glial neoplasm. Next-generation sequencing confirmed the presence of fusion between the FET and cAMP response element-binding (CREB) family of genes. This fusion is diagnostic of IMMT, with only 19 such cases reported so far. The authors would like to highlight the need for genomic sequencing for the diagnosis of this tumor, its propensity to recur locally, and its relatively better prognosis as compared to high-grade gliomas.

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.

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.

Extramedullary Hematopoiesis Presenting with Thoracic Spinal Cord Compression in a Young Adult with Thalassemia Major: A Case Report.

Background: Extramedullary hematopoiesis (EMH) refers to the production of blood cellular components at sites other than the bone marrow, namely liver, spleen, and lymph nodes. The common sites associated with this condition are the liver, spleen, and lymph nodes whereas the common conditions associated with it are myelofibrosis, myelodysplasia, thalassemia, sickle cell anemia, and polycythemia vera. Case Description: This report describes a young male with thalassemia major, who presented with symptomatic cord compression due to a thoracic intraspinal lesion. It was surgically excised and diagnosed as a case of EMH. The boy recovered fully and has been asymptomatic for six months now. Conclusion: The occurrence of EMH in the thoracic spine is uncommon, whereas symptomatic cord compression as a result of it is even more unusual. Magnetic resonance imaging (MRI) is the diagnostic imaging of choice and treatment options that can be offered are surgical decompression, radiotherapy, hydroxyurea, and transfusion of packed red blood cells (RBCs).

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.

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.

Computed Tomography Evaluation of Craniovertebral Junction in Asymptomatic Central Rural Indian Population.

Objectives Craniovertebral junction (CVJ) is a unique, mobile and complicated component of our spine which necessitates specific study pertaining to its structure as well as pathologies. This study aims to report the normative data detailing the CVJ anatomy among the rural population of Central India which would help us in understanding the joint dynamics. Materials and Methods A retrospective observational study was undertaken in the Department of Neurosurgery and Radiology, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Maharashtra, from December 2018 to May 2019. A total of 255 head injury patients with a normal CT brain cervical spine were included in this study. Anterior and posterior atlantodental interval (AADI/PADI), clivus length (CL), foramen magnum diameter (FMD), Boogard's and basal angle (BOO & BA) were measured and analyzed. Statistical Analysis Statistical analysis was done using Microsoft Excel 2016. A web-based, open source application known as OpenEpi.com (version 3.01) was used for applying unpaired t -test. Results This study had a mean age of 42.9 years. The difference in mean value of AADI between male and female population was not found to be statistically significant, while in case of PADI, CL, FMD, BOO and BA, it was found to be significant ( p < 0.05). On comparison of cases with age ≤ 20 years and > 20 years, we found the difference in values of ADI, CL and FMD to be statistically significant ( p < 0.05). Conclusion Although there are some similarities, namely, AADI measurements as compared with other studies, there are differences in cutoff values of other parameters. Being a major draining reference center for rural population in India, this data can be extrapolated to a similar population for reference.

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.

Evaluation of the Evan's and Bicaudate Index for Rural Population in Central India using Computed Tomography.

INTRODUCTION: Evans index (EI) and Bicaudate index (BCI) are practical markers of ventricular volume and are helpful radiological markers in the diagnosis of normal pressure hydrocephalus. Worldwide, variation exists in normative studies for both these indices. Most of the studies conducted for EI and BCI are based on the Western population data. No study has been performed on the rural population of Central India. The purpose of this study is to develop normative data on EI and BCI that can be extrapolated for future reference. MATERIALS AND METHODS: This was a retrospective study conducted from December 2018 to May 2019 in MGIMS Hospital, Sevagram, Maharashtra, India, which is a rural hospital in Central India. All patients with either a head injury or neurological complaints although with normal computed tomography (CT) brain were included in the study. Patients with diagnosed neurological disorder, clinical features suggesting hydrocephalus, or intracranial pathology on CT brain were excluded from the study. Five hundred and eleven patients were selected for this study, and EI and BCI was calculated for them. RESULTS: The mean value of EI and BCI in our study was 0.2707 and 0.1121, respectively. Both indices showed a statistically significant difference between males and females. The value of both indices increased with age. CONCLUSION: Although our study is in agreement with the cutoff value of EI to diagnose dilated lateral ventricles as 0.3 for age <70 years, cutoff value of EI for the older population should be reconsidered to 0.34.

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.

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.

De novo Choledocholithiasis in Retained Common Bile Duct Stent.

De novo choledocholithiasis means formation of stone in the common bile duct (CBD). It can present as biliary colic, jaundice, cholangitis, pancreatitis or it may be asymptomatic. There are various indications for biliary stenting like CBD stone, CBD stricture, biliary leak, peri ampullary carcinoma, CBD malignancy, etc. Foreign bodies like silk sutures, endo-clips, fish bone, retained T- tubes, plastic or metallic stents, etc. lead to biliary stasis leading to eventual stone formation. Here, we discuss a case of choledocholithiasis post-cholecystectomy with CBD stenting done 15 years back which had migrated and acted as a nidus for stone formation in the CBD and hepatic duct.