Factors That Predict Recurrence Following Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea.

BACKGROUND: The endoscopic approach has gained popularity in cerebrospinal fluid (CSF) rhinorrhea repair with high success rates, yet recurrence is frequent. We analyzed our cases to determine the outcomes of endoscopic repair of CSF rhinorrhea and the effect of several perioperative factors on the success of repair. METHODS: A retrospective review of 50 patients who underwent endoscopic repair of CSF rhinorrhea between January 2013 and July 2023 was performed, collecting details of presentation, surgery, and postoperative period. RESULTS: The most frequent etiology was nontraumatic CSF rhinorrhea (76%), in which the defect was most commonly located at the left cribriform plate, followed by traumatic CSF rhinorrhea (24%), in which sphenoid defects were most frequent. Traumatic CSF rhinorrhea was more common among male patients and was significantly associated with anosmia. Success rate at first repair attempt was 84%. Persistent CSF rhinorrhea was present in 3 patients (6%), and 5 patients (10%) developed recurrence of CSF rhinorrhea. Overall, 7 patients required reoperation, with 100% success rate after the second surgery. The use of 3-layered repair with fat, fascia lata, and mucosal flap was protective against repair failure, whereas bilateral defects and duration of symptoms >1 year were significantly associated with repair failure. The use of lumbar drain did not demonstrate a difference in repair success rate. CONCLUSIONS: Endoscopic repair of CSF rhinorrhea appears to be safe and effective when performed with accurate localization of the site of the lesion and multilayered repair. Potential predictors of recurrence include bilateral and long-standing defects.

Factors predicting recurrence in benign spinal nerve sheath tumors: A retrospective study of 457 patients from a single institution.

BACKGROUND: Benign Nerve sheath tumors (NST) comprise almost one-third of primary spinal tumours. The majority are sporadic. They have low rates of recurrence but an occasional recurrence may need re-surgery. The present study was designed to identify the variables that can predict the risk of their recurrence. METHODS: A retrospective chart review was done including all the histologically proven benign spinal NSTs operated between 2001 and 2019 in our institute. Demographic, operative and postoperative follow-up data were recorded. Recurrence was defined as local reappearance after definite surgical excision or symptomatic increase in size of a residual tumour on follow-up imaging studies. Statistical analysis was done to determine the significant variables associated with local recurrence. RESULTS: 457 patients with a median age of 38 years operated for 459 NSTs qualified for the study. The most frequent location of occurrence of tumours was found to be Low Cervical level (C3-C7 levels). Majority of Schwannoma were located intradurally while Neurofibroma were dumb-bell shaped and extradural. Most of the tumours had solid consistency. Post operatively, 7.7% patients developed complications. 7.8% tumours developed local recurrence after median period of 12 months. The patients developing recurrence were younger compared to nonrecurring tumors. On univariate analysis, male gender, Low cervical and Cervicothoracic junction location were associated with higher recurrence. On multivariate analysis, location at Cervicothoracic junction reached significance. CONCLUSION: Overall recurrence risk among all NST was 7.8% with a median progression free survival of 36 months. The location of tumour at cervicothoracic location was the significant risk factors for recurrence of tumour in our study.

Gamma Knife Radiosurgery in Partially Embolised Arteriovenous Malformations: Management Dilemmas and Outcomes.

Introduction: The role of Gamma Knife radiosurgery (GKRS) in partially embolized arteriovenous malformations (AVMs) has always remained a subject of debate. The aim of this study was to evaluate the efficacy of GKRS in partially embolized AVMs and to analyze factor that influence its obliteration. Methods: This was a retrospective study from a single institute performed over a period of 12 years (2005-2017). It included all patients who underwent GKRS for partially embolized AVMs. Demographic characteristics, treatment profiles, and clinical and radiological data were obtained during treatment and follow-up. Obliteration rates and factors affecting the same were sought and analyzed. Results: A total of 46 patients with a mean age of 30 years (range: 9-60 years) were included in the study. Follow-up imaging was available for 35 patients either by digital subtraction angiography (DSA) or magnetic resonance imaging (MRI). We found complete AVM obliteration in 21 patients (60%): one had near total obliteration (>90% obliteration), 12 had subtotal obliteration (<90%), and one had no change in the volume following GKRS. Following embolization alone, an average of 67% of the AVM volume was obliterated which resulted in an average 79% final obliteration rate after Gamma Knife radiosurgery. Mean duration to complete obliteration was found to be 3.45 years (range: 1-10 years). There was a significant difference (P = 0.04) in the mean interval between embolization and GKRS among cases with complete obliteration (12 months) and those with incomplete obliteration (36 months). There was no significant difference (P = 0.49) in the average obliteration rate between the ARUBA-eligible unruptured AVMs (79.22%) and ruptured AVMs (79.04%). Bleeding after GKRS during the latency period had a negative impact on obliteration (P = 0.05). Other factors like age, sex, Spetzler-Martin (SM)-grade, Pollock Flickinger score (PF-score), nidus volume, radiation dose, or presentation before embolization had no significant influence on obliteration. Three patients had permanent neurological deficits after embolization and none after radiosurgery. Six out of nine patients (66%) presenting with seizures were seizure-free after the treatment. Hemorrhage was noted in three patients following combined treatment and were managed non-surgically. Conclusion: Obliteration rates in partially embolized AVM after Gamma Knife are inferior when compared to Gamma Knife alone; moreover with volume staging and/or dose staging being increasingly plausible due to the new ICON machine, embolization may be completely replaced. However we have shown that in complicated and carefully chosen AVMs, embolization followed by GKRS is a valid modality of management. This study represents a real-world picture of individualized AVM treatment depending on patient choices and resources available.

Chitinase 3-Like 2.

OBJECTIVES: We aimed to evaluate the expression pattern of chitinase 3-like 2 (CHI3L2) in the tumor core and peritumoral brain zone (PBZ) of newly diagnosed glioblastoma (GBM) in recurrent tumors and its association with patient prognosis. METHODS: The study was conducted on three sample sets derived from different patient cohorts. Messenger RNA (mRNA) expression of CHI3L2 in the tumor core and PBZ (n = 34) compared with control (n = 20) tissues was studied by quantitative polymerase chain reaction in sample set 1. Sample set 2 included 19 paired, primary-recurrent GBM tissues. Sample set 3 comprised 82 GBM tissues of patients with treatment and follow-up information. Immunohistochemistry (IHC) was performed on all three sample sets. RESULTS: mRNA expression of CHI3L2 was significantly higher in the tumor core and PBZ compared with control (P < .0001). By IHC, CHI3L2 showed strong cytoplasmic staining in tumor cells. Recurrent tumors had a higher expression of CHI3L2 compared with primary tumors (P = .007). Survival analysis showed CHI3L2 expression was associated with shorter overall survival (P = .034) and progression-free survival (P = .010), which was in line with The Cancer Genome Atlas cohort (P = .043). CONCLUSIONS: High expression of CHI3L2 in the tumor core and PBZ, as well as its association with tumor recurrence and poor patient prognosis, suggests it might be contributing to tumor spread and recurrence.

Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma.

Objective Malignant peripheral nerve sheath tumor (MPNST) arises from nerve sheaths, mostly seen in peripheral nerves but rare in craniospinal nerves. The information available in the literature to build up treatment strategy and improve clinical outcomes is scarce. We are reviewing cases from our institute, with emphasis on radiological features for early differentiation from its benign variants. Methods We analyzed pathologically diagnosed cases retrospectively from January 2007 to December 2018 at our institute. Clinicoradiological details and treatment parameters were collected from medical records for evaluation. Each case was contacted telephonically for final clinical follow-up at the time of writing the manuscript. Results A total of seven cases of MPNST were diagnosed in the last 10 years. It included four intracranial and three spinal cases. The mean age for the cohort was 34.3 years, with five females. We could achieve gross total resection (GTR) and subtotal resection in four (57.1%) and two (28.6%) cases, respectively. We could achieve an overall survival of 57.1% in the average follow-up of 28.2 months (range: 8-84 months). Conclusion MPNST is a rare tumor with a bad prognosis. Radical surgical resection is the mainstay of the treatment, but it is not always possible to achieve it because of the inaccessible location and large size of lesions. Preoperative diagnosis is challenging; however, few radiological findings may give a clue toward it. As a disease entity overall, it has a poor outcome with a high rate of fatality.

What Happens after Therapy? Quality of Life and Neurocognitive Functions of Children with Malignant Posterior Fossa Tumors after Adjuvant Therapy.

PURPOSE: The health-related quality of life (HRQoL) is an important endpoint in modern clinical practice with improved survival of pediatric posterior fossa malignant brain tumors (PFMBTs). We evaluated the effect of environmental and psychosocial milieu on QoL and cognitive functioning (CF) of Indian children with PFMBT. METHODS: In a cross-sectional study, 47 children <18 years of age with medulloblastoma or anaplastic ependymoma were evaluated ≥6 months after completion of adjuvant therapy. All clinical and socioeconomic details, educational status of child and family members, socioeconomic status, environmental factors affecting QoL were documented. Children underwent HRQoL evaluation using Pediatric quality of life Inventory (PedsQL) questionnaire and neuropsychological evaluation. RESULTS: The median age of the cohort at presentation was 7 years (1-18) and median duration of evaluation after adjuvant therapy was 16 months. In 47 families, 72.34% had low monthly income and 76.6% of mothers took formal education. QoL scores were above median values. Parents reported scores highlighted that Lansky performance score (P = 0.001) and maternal education (P = 0.043) significantly influenced the cognitive component of QoL. Twenty-seven children had below-average IQ. Young age at presentation (P = 0.020), maternal education (P = 0.032), high socioeconomic status (P = 0.001) influenced the IQ score. Even though the majority of children (57.44%) had below-average IQ, they had a score of more than 50 on the cognitive functioning scale. A total of 72.5% of the eligible children in our cohort went back to school following therapy, though often with a delay of one academic year. CONCLUSIONS: Overall cognitive functioning scores of these children are good, but they are not representative of actual neurocognitive tasks based performance or IQ scores. Children should remain under regular follow-up with a neurocognitive assessment and psychological counseling at regular intervals.

Anterior Odontoid Screw Fixation for C2 Fractures: Surgical Nuances, Complications, and Factors Affecting Fracture Union.

OBJECTIVE: Type II odontoid fractures are the commonest C2 fractures. The management of dens fractures remains controversial with various radiological and fracture morphological factors affecting the approach and outcomes. METHODS: All cases of anterior odontoid screw fixation between 2010 and 2020 were retrospectively analyzed. Patients' clinical, radiological (type of fracture, orientation, displacement, and diastasis), operative (single vs. double screw) and follow-up records were documented. The postoperative imaging findings were classified into grades I (excellent), II (good), and III (poor) based on the anatomical alignment with the screw. Follow-up cervical computed tomography was reviewed for fracture union. RESULTS: A total of 49 patients with a mean age of 37.3 ± 13.8 years were included in the study. The average time from injury to surgery was 23.1 ± 22.2 days. The bicortical screw purchase and superoposterior odontoid tip breach significantly affected the postoperative alignment (P = 0.035 each). Fracture union was noted in 41 (83.7%) patients. The horizontal fracture displacement ≥2.5 mm (P = 0.02) and poor postoperative fracture alignment (P < 0.001) resulted in worse fracture union rates. Transient dysphagia was the commonest complication (n = 12), whereas screw breakout (n = 2) and pullout (n = 2) were other hardware-related complications. One patient underwent re-exploration for screw readjustment and 2 underwent posterior instrumentation. CONCLUSIONS: Anterior odontoid screw fixation is technically straightforward, less morbid, and preserves biomechanical cervical mobility, when compared with the posterior instrumentation. With careful patient selection, meticulous surgical planning, and intraoperative image-guided screw insertion, good fracture union outcomes can be obtained. In the current study, we were able to achieve stable fracture union in 83.7% patients.

Transcranial approach for pituitary adenomas - An evaluation of surgical approaches over two decades and factors influencing peri-operative morbidity.

PURPOSE: Transcranial surgery(TCS) for pituitary adenoma(PA) is mostly reserved for lesions with widespread extensions. We sought to analyse the intraoperative challenges, morbidity and the factors associated with morbidity of transcranial approaches, in a large series from a tertiary neurosurgical institute. METHODS: We retrospectively evaluated 137 patients who underwent TCS for PA in our institute. The details of their clinical features, imaging, operative and peri-operative events and complications were collected and analysed. The factors associated with morbidity were evaluated. RESULTS: The mean age of the cohort was 40.86 ± 13.35 years. 21 (15.3%) patients developed significant post-operative hematoma, out of which 7 patients (5.1%) required re-exploration. Post-operative diabetes insipidus was noted in 45 patients (32.84%), while SIADH was noted in 10 patients (7.35%). Other complications were cranial nerve palsy in 14 patients (10.2%), visual deterioration in 7 patients (5.1%). Neither tumour characteristics nor the choice of approach was associated with occurrence of post op hematoma. SIADH was significantly associated with patients with tumours involving cavernous sinus (p = 0.019) and subfrontal extension (p = 0.031). Patients with post-operative hematoma had significantly higher incidence of post-op DI (57.1% vs 28.7%; p = 0.021), while similar correlation was not noticed with SIADH (4.7 vs 7.8%). CONCLUSION: TCS plays a distinct, albeit a small role in surgical management of pituitary adenoma. Tumour related or approach related factors are not significantly associated with the incidence of hematoma. Post-operative hematoma significantly influences the incidence of DI. A proper and judicious selection of approach and meticulous surgical technique should result in a reduction in associated morbidity and mortality.

Diagnostic Utility of CUSA Specimen in Histopathological Evaluation of Tumors of Central Nervous System.

INTRODUCTION: Cavitron Ultrasonic Surgical Aspirator (CUSA) is a technique used for the surgical treatment of tumors that aids the surgeon in highly selective tumor sampling with minimal injury to surrounding tissues. The utility of the tissue obtained from CUSA for histopathological diagnosis of central nervous system tumors is not as well-known as its surgical benefits. Even though a few studies have evaluated the diagnostic accuracy of CUSA specimen, these have dealt with very few cases. METHODOLOGY: In this study, we nil analysed 73 cases of CNS tumors (glial and non-glial) where CUSA specimen was available for histopathological examination and compared with findings on conventional samples as gold standard. RESULTS: Most frequent types of artefacts induced by CUSA included tissue breakdown resembling necrosis, empty spaces in tissues, and crush artefacts particularly in cellular tumors, that interfered with interpretation. CUSA samples were found optimal for diagnosis of non-glial tumors (45/73), (mainly mesenchymal), wherein the diagnostic utility was comparable to the conventional samples. Difficulties were encountered in glial neoplasms, medulloblastomas and meningiomas. In glial neoplasms (28/73), accurate grading was not possible (9/28, 32%) utilising CUSA samples alone as necrosis and mitosis were not represented. Similarly in meningiomas, mitosis and brain invasion, essential for grading, was not recognizable in CUSA samples. In medulloblastomas, extensive crush artefacts interfered with diagnosis and histological subtyping making it mandatory to examine conventional tissue samples and CUSA. Immunohistochemistry results were optimal with CUSA tissue, wherever performed. CONCLUSION: The greatest benefits of CUSA, is its ability to sample multiple areas enhancing the yield in heterogenous tumors like gliosarcomas and its utility in tumors at surgically inaccessible sites. As a policy, we recommend that it is beneficial that all surgically excised tissues including those from the CUSA bottle and suction be sent for histopathological analysis for optimising diagnostic accuracy.

Low mitochondrial DNA copy number is associated with poor prognosis and treatment resistance in glioblastoma.

INTRODUCTION: Mitochondrial DNA (mtDNA) content in several solid tumors was found to be lower than in their normal counterparts. However, there is paucity of literature on the clinical significance of mtDNA content in glioblastoma and its effect on treatment response. Hence, we studied the prognostic significance of mtDNA content in glioblastoma tumor tissue and the effect of mtDNA depletion in glioblastoma cells on response to treatment. MATERIALS AND METHODS: 130 newly diagnosed glioblastomas, 32 paired newly diagnosed and recurrent glioblastomas and 35 non-neoplastic brain tissues were utilized for the study. mtDNA content in the patient tumor tissue was assessed and compared with known biomarkers and patient survival. mtDNA was chemically depleted in malignant glioma cell lines, U87, LN229. The biology and treatment response of parent and depleted cells were compared. RESULTS: Lower range of mtDNA copy number in glioblastoma was associated with poor overall survival (p = 0.01), progression free survival (p = 0.04) and also with wild type IDH (p = 0.02). In recurrent glioblastoma, mtDNA copy number was higher than newly diagnosed glioblastoma in the patients who received RT (p = 0.01). mtDNA depleted U87 and LN229 cells showed higher survival fraction post radiation exposure when compared to parent lines. The IC50 of TMZ was also higher for mtDNA depleted U87 and LN229 cells. The depleted cells formed more neurospheres than their parent counterparts, thus showing increased stemness of mtDNA depleted cells. CONCLUSION: Low mtDNA copy number in glioblastoma is associated with poor patient survival and treatment resistance in cell lines possibly by impacting stemness of the glioblastoma cells.

Endoscopic intervention for intraventricular neurocysticercal cyst: Challenges and outcome analysis from a single institute experience.

OBJECTIVE: Endoscopic intervention is presently proposed as standard approach for the treatment of Intraventricular neurocysticercal cyst (IVNCC) as it helps to retrieve the cyst as well as CSF diversion. We present our series of 61 patients with IVNCC managed by endoscopic intervention and analyze the outcome. METHOD: A retrospective analysis of 61 patients with IVNCC managed between 1998-2019 at our institute was performed. We reviewed the clinical details of consecutive patients, management, and outcome. RESULTS: There were 61 patients with 34 males and 27 females. The mean age was 25 years. Fourth ventricular location is the most common (n = 34) followed by third ventricle(n = 14) and lateral ventricle (n = 13). Cyst retrieval could be done in 43 cases, while the cyst could not be retrieved in 18 cases due to intraventricular bleed, CSF turbidity, adhesion of cyst wall etc. Along with cyst retrieval, some patients underwent Endoscopic Third ventriculostomy, septostomy, foraminotomy for internal CSF diversion. Seven patients had a preoperative VP shunt surgery. The median follow-up was 12 months. Preoperative shunt (CI:1.33-62, P = 0.02) was associated with failure of cyst retrieval in univariate analysis as well as in multivariate regression analysis (CI: 0.02-0.94, P = 0.04). Two patients underwent shunt surgery at follow-up period due to the failure of endoscopic CSF diversion. CONCLUSION: Endoscopic management of IVNCC is a safe and effective management option, avoiding an indwelling shunt system. Endoscopic third ventriculostomy should be considered for patients with IVNCC and hydrocephalus.

Proteomic profiling of medulloblastoma reveals novel proteins differentially expressed within each molecular subgroup.

OBJECTIVES: The objective of the study was to identify novel medulloblastoma (MB) biomarkers through proteomic profiling, correlate it with the molecular subgroups of MB and assess the clinical significance. METHODS: Archived paraffin embedded tumor tissue blocks from 118 MB patients, operated at our institute were retrieved. Clinical information was documented from the hospital database. Tumours were stratified into molecular subgroups using the IHC markers- ß Catenin, GAB-1, YAP-1 and p53. Six fresh MB tumour tissues and two control cerebellar tissues were subjected to proteomic profiling to study differential protein expression in molecular subgroups using high resolution mass spectrometry. Prominent signalling pathways activated in each subgroup were identified using the Panther pathway software. RESULTS: Non WNT/SHH group was the most common (61.1 %), followed by SHH and WNT. p53 immunopositivity did not correlate with prognosis in any subgroup. Proteomic profiling revealed several novel proteins differentially expressed between MB molecular subgroups. Signalling pathways exclusively enriched in each molecular subgroup were also identified. The top upregulated proteins were PMEL and FBN2 in the WNT subgroup, SYNGR2 in the SHH subgroup and GFAP, IMPG2 and MAGEA10 in the Non WNT/Non SHH group. We validated GFAP by immunohistochemistry on the archived samples (n = 118) and noted two types of staining pattern in MBs - reactive (stellate) astrocytes and tumour cell staining. GFAP immunopositivity in tumor cells of SHH subgroup correlated with a better prognosis. CONCLUSIONS: Proteomic profile identified several novel proteins differentially regulated within the molecular subgroups that could serve as potential diagnostic /prognostic biomarkers. Notably, GFAP, which was derived from proteomics data, when validated by IHC, revealed a variable staining pattern in MB tumours. The prognostic significance of GFAP in SHH tumor patients further points at the heterogeneity of this subgroup. The study also throws light on the signaling pathways activated in MB and in turn its plausible role in the tumorigenesis.

Surgical Management and Outcomes of Aneurysms of Posterior Inferior Cerebellar Artery: Location-Based Approaches with Review of Literature.

Background Posterior inferior cerebellar artery (PICA) is a tortuous, variable, and uncommon site for aneurysms. Surgical management of PICA aneurysms involves careful selection of approach based on the location of the aneurysm and meticulous dissection of the neurovascular structures and perforators. Materials and Methods We did a retrospective review of all the PICA aneurysms operated at our institute in the past 10 years along with the site, presentation, and approach used for the same. Preoperative World Federation of Neurosurgical Society scores and follow-up modified Rankin scores (mRS) were also evaluated. During the same period, data for intervention cases of PICA aneurysm were also collected with follow-ups for a comparative analysis. Results A total of 20 patients with 21 PICA aneurysms were reviewed. All the reviewed cases presented with subarachnoid hemorrhage, and the most common location was the lateral medullary segment and vertebral artery (VA)-PICA junction. Midline approaches were used for distal PICA cases, with far-lateral approach reserved for anterior medullary/VA-PICA junction. No lower cranial nerve palsies were recorded at follow-up. Four cases needed cerebrospinal fluid diversion and two developed cerebellar infarcts. All cases were mRS 0 to 2 at follow-up. Conclusion Our series compares well with some of the larger surgical series of PICA aneurysms. This may be due to early referral patterns and early surgery (<24 hours) policy at our institution. Anatomical knowledge of PICA anatomy and sound perioperative management are keys to good outcomes in these cases.

CSF Rhinorrhea Following Medical Treatment for Prolactinoma: Management and Challenges.

Objective Cerebrospinal fluid (CSF) rhinorrhea following medical management of prolactinoma is a rare complication. We evaluated the clinical background of this condition, identify potential risk factors, and discuss the management options and challenges involved. Methodology We retrospectively reviewed clinical details of patients who were treated for CSF leaks among patients treated for prolactinoma between 2013 and 2017. Results Seven patients were treated for CSF rhinorrhea in the context of prolactinoma, with the age range between 24 and 56 years. Six patients had CSF leak following initiation of cabergoline, while one patient presented with CSF rhinorrhea. The time of onset of leak following medical treatment ranged from 14 days to 5 years. The mean preoperative serum prolactin level was 12,638 ng/mL (range: 1,000-26,287 ng/mL). All patients underwent repair of skull base defect. (four endoscopic, two microscopic, and one bifrontal craniotomy). The site of defect in the majority of patients was the sellar floor. Two patients who were initially managed with acetazolamide alone, eventually required surgical repair. Three patients were cured of CSF leak with a single procedure. Three patients had to undergo re-exploration and endoscopic repair after their first surgery failed. Two patients required lumboperitoneal (LP) shunt after a failed endoscopic transsphenoidal repair. Conclusion Surgical management for medically-induced CSF rhinorrhea is necessary; however, it can pose significant issues. Endoscopic repair of the defect should be considered at the earliest. Multiple surgical procedures are often required because of skull base erosion. LP shunt can be considered if CSF leak persists following multiple surgeries.

Microvascular Anastomosis Using Only 2-Throw Reef-Knots: A Technical Note.

In macrosurgery creating a knot with at least three throws is an established practice. The potential disadvantages of this practice in microsurgery include the following: the direction of the cut ends interfere with the suture line, unbalanced nature of knot disturbs the apposition of delicate vessel ends and the excessive knot weight. A reef knot with only 2 throws may thus be a better alternative. We have described our technique of end to end anastomosis with 2-throw reef knots in a rat femoral artery model using one way up method. Judging from the surgical outcome the practice of two throw reef knot seems feasible and appealing.

Multiple-site neural tube defects: embryogenesis with complete review of existing literature.

OBJECTIVE: Multiple-site neural tube defects (MNTDs) are very uncommon, with the predominant number of cases being reported in developing countries. The classic theory of neural tube closure fails to explain the occurrence of these defects. Multisite closure theory, first proposed in 1995, explains most of the occurrences with a few modifications specific to a few defects. In this paper, the authors endeavor to explain all the defects, along with their genetic and embryological bases, and to review the available literature and discuss their own experience in the management of these complex cases. METHODS: The authors retrospectively reviewed the data of all the patients treated surgically for MNTDs over that past 14 years. All possible demographic data, clinical details, and radiological imaging data were reviewed. In addition, surgical parameters, complications, and status at follow-up of more than 12 months were evaluated. All previously reported cases of MNTD were analyzed, and comparisons with the present series were made. RESULTS: A total of 3 major series (including the present one) on MNTDs have been from India. A total of 57 such cases (including those of the present series) have been reported in the available literature. While previous series reported a higher incidence of spinal defects, the present series had a higher rate of cephalic defects (55%). Among the reported cases, insertion of a ventriculoperitoneal shunt was necessary in 12 (26%), and only 4 patients were operated on in 2 stages. Neurological status at presentation dictated outcome. CONCLUSIONS: MNTDs are extremely rare, and their embryogenesis is different from that of single neural tube defects. Simultaneous repair of 2 or even 3 defects is possible in a single-stage surgery. The requirement of a shunt is uncommon, and complications following surgery are rare. Folic acid supplementation may reduce the incidence of defects.

Medulloblastoma: Distinctive Histo-Molecular Correlation with Clinical Profile, Radiologic Characteristics, and Surgical Outcome.

OBJECTIVE: Medulloblastoma (MB) is a heterogenous tumor, and the prognosis is influenced by various clinical, histological, and molecular factors. The aim of the study is to determine the clinical profile and radiologic characteristics among the histo-molecular subgroups, the predictors of surgical outcome, and the pattern of relapse in pediatric and adult MB. METHOD: An analysis of 118 patients of MB who underwent surgical treatment at National Institute of Mental Health and Neurosciences, India, over a 7-year period (2005-2011) is presented. The clinical profile, radiologic characteristics, surgical nuances, and survival patterns are discussed. The relevant statistical analysis was done using SPSS software, version 22.0. RESULTS: The mean age of the cohort was 12 years (12.3 ± 8.7). The primary manifestation was raised intracranial tension headache in 53 patients (44.9%), which was the predominant symptom in large cell/anaplastic (LCA)- and WNT-activated subgroups. The median preoperative Karnofsky performance score was 60 (60.6 ± 12.9). Vermian and hemispheric location of tumor was most commonly observed in non-WNT/non-SHH (groups 3 and 4; 91.7%) and SHH-activated (42.9%) subgroups, respectively. Ninety-two patients (78%) underwent preoperative ventriculoperitoneal shunts (VPS) for obstructive hydrocephalus (HCP) and 14 patients (11.8%) underwent VPS in the postoperative period. The median overall survival (OS) for the whole group was 82.1 ± 5.7 months and the median recurrence-free survival was 51.0 ± 4.8 months. While radiotherapy had a significant influence on OS, progression-free survival was influenced by radiotherapy as well as chemotherapy in both pediatric and adult cohort. Desmoplastic/nodular subtype and WNT-activated subgroup had the best prognosis; LCA and non-WNT/non-SHH had the worst prognosis. CONCLUSIONS: Majority of the patients were pediatric in the study. Age, hemispheric location of tumor, extent of resection, and adjuvant treatment status were the important clinical prognostic factors for survival. Surgery for MB is formidable, and VPS can be considered in persistent symptomatic and progressive HCP. Our study on pediatric and adult MB validates the prognostic significance of various clinical, radiologic, and histo-molecular parameters of MB.

Gene Expression in Intracranial Aneurysms-Comparison Analysis of Aneurysmal Walls and Extracranial Arteries with Real-Time Polymerase Chain Reaction and Immunohistochemistry.

BACKGROUND: This study was aimed at evaluating the gene expression levels of 4 genes in the intracranial aneurysm wall and comparing them with extracranial arteries. The analysis was done using real-time polymerase chain reaction (RT-PCR) and immunohistochemistry (IHC). Also, a correlation of the differential genetic expression was done with various patient clinical and radiologic factors. METHODS: The quantitative assessment of ribonucleic acid levels was done with RT-PCR and was validated with IHC. The genes studied were collagen 1A2 (COL1A2), tissue inhibitor of metalloproteinase 4 (TIMP4), cathepsin B (CTSB), and alpha-1 antitrypsin (α-1 AT). The analysis was done on 24 aneurysm sacs and superficial temporal/occipital artery samples from patients undergoing surgical clipping. RESULTS: The mean fold change of COL1A2 in the aneurysm sample was 8.89, that of TIMP4 was 10.16, that of CTSB was 1.02, and that of α-1 AT was 1.46 when compared with normal control vessel on PCR. On semiquantitative IHC, COL1A2 was 94.44%, α-1 AT was 77.8% overexpressed, CTSB was positive in 50%, and the expression of TIMP4 was 94.4% underexpressed in aneurysmal walls. There was no statistically significant correlation between patient profile and gene expression. CONCLUSIONS: On RT-PCR and IHC analysis, COL1A2 and α-1 AT were overexpressed, CTSB was marginally overexpressed, and TIMP4 had equivocal expression in the aneurysmal sac when compared with the normal extracranial vessel. This is the first study of its kind in the Indian population with the largest sample size on live human patients.

Intracranial fungal granuloma: a single-institute study of 90 cases over 18 years.

OBJECTIVE: Intracranial fungal granuloma (IFG) remains an uncommon entity. The authors report a single-institute study of 90 cases of IFG, which is the largest study until now. METHODS: In this retrospective study, all cases of IFG surgically treated in the years 2001-2018 were included. Data were obtained from the medical records and the pathology, microbiology, and radiology departments. All relevant clinical data, imaging characteristics, surgical procedure performed, perioperative findings, and follow-up data were recorded from the case files. Telephonic follow-up was also performed for a few patients to find out their current status. RESULTS: A total of 90 cases consisting of 64 males (71.1%) and 26 (28.9%) females were evaluated. The mean patient age was 40.2 years (range 1-79 years). Headache (54 patients) was the most common presenting complaint, followed by visual symptoms (35 patients), fever (21 patients), and others such as limb weakness (13 patients) or seizure (9 patients). Cranial nerve involvement was the most common sign (47 patients), followed by motor deficit (22 patients) and papilledema (7 patients). The mean duration of symptoms before presentation was 6.4 months (range 0.06-48 months). Thirty patients (33.3%) had predisposing factors like diabetes mellitus, tuberculosis, or other immunocompromised status. A pure intracranial location of the IFG was seen in 49 cases (54.4%), whereas rhinocerebral or paranasal sinus involvement was seen in 41 cases (45.6%). Open surgery, that is, craniotomy and decompression, was performed in 55 cases, endoscopic biopsy was done in 30 cases, and stereotactic biopsy was performed in 5 cases. Aspergilloma (43 patients) was the most common fungal mass, followed by zygomycosis (13 patients), chromomycosis (9 patients), cryptococcoma (7 patients), mucormycosis (5 patients), and candida infection (1 patient). In 12 cases, the exact fungal phenotype could not be identified. Follow-up was available for 69/90 patients (76.7%). The mean duration of the follow-up was 37.97 months (range 3-144 months). The mortality rate was 52.2% (36/69 patients) among the patients with available follow-up. CONCLUSIONS: A high index of suspicion for IFG should exist for patients with an immunocompromised status and diabetic patients with rhinocerebral mass lesions. Early diagnosis, aggressive surgical decompression, and a course of promptly initiated antifungal therapy are associated with a better prognosis.