Postoperative Seizure Control in Adult Diffuse Insular Gliomas Presenting with Seizures: A Retrospective Single-Center Experience and Proposal of a Novel Risk Scoring System.

Background: Studies on insular gliomas (IGs) generally focus on the oncological endpoints with a relative scarcity of literature focusing on the seizure outcomes. Objectives: To study the predictors of long-term postoperative seizure control in IG and propose a novel risk scoring system. Methods: Histopathologically proven, newly diagnosed adult IGs (>18 years) operated over a 10-year period were studied for postoperative seizure control as per International League Against Epilepsy (ILAE) grades at 6 weeks and at last follow-up (minimum of 6 months, median 27 months). Logistic regression analysis was performed and regression coefficients with nearest integers were used to build a risk prediction model. Receiver operator curve (ROC) analysis determined the predictive accuracy of this model. Results: The 6-week postoperative seizure freedom dropped to 41% at the last follow-up. The seizure-free group lived longer (100.69 months, 95% CI = 84.3-116.99 (60%)) than those with persistent postoperative seizures (27.92 months, 95% CI = 14.99-40.86). Statistically significant predictors (preoperative seizure control status, extent of resection, tumor extension to temporal lobe, and lack of postoperative adjuvant therapy) were used to compute a risk score, the score ranging from 0 to 9. A score of four most optimally distinguished the risk of postoperative seizures with an area under the ROC of 91.4% (95% CI: 84.1%, 98.7%, P < 0.001). Conclusion: In our experience, around 60% of patients obtained seizure freedom after surgery, which reduces over time. Control of seizures paralleled survival outcomes. Our proposed scoring system may help tailor management strategies for these patients.

Looking Beyond the Anterolateral Skull Base with Frontotemporal Dural Fold Unlocking and Extradural Clinoidectomy: A Series of 17 Cases Deciphering the Translation from a Cadaver to Real, and Real to Pathologically Distorted Anatomy.

Objective: This article aims to discuss the surgical nuances and major adjustments necessary in unlocking the frontotemporal dural fold (FTDF) and extradural anterior clinoidectomy (EDAC) in actual cases, allowing translation from the cadaveric to a clinical scenario. Materials and Methods: We retrospectively reviewed the technical details of 17 procedures over 8 years, where both the initial steps (FTDF unlocking and EDAC) were performed. Lesions involving or extending to the anterolateral skull base, like the suprasellar cistern, optico-carotid cistern, interpeduncular cistern, petrous apex, and cavernous sinus, were included. The clinical data of the patients were retrieved retrospectively from the hospital information system (HIS) and in-patient records. This study was approved as a multicenter individual project with IEC No: 2020-342-IP-EXP-34. Results: An illustrated note of the common steps and outcome of the 17 procedures of unlocking the FTDF and EDAC done is presented. The technique provided adequate exposure in performing aneurysmal clipping (posterior communicating artery [P. com], basilar top, and superior hypophyseal artery [SHA] aneurysm), giant pituitary adenoma (Wilson Hardy grade 4E, n = 2), fifth nerve schwannoma (n = 4), right Meckel's cave melanoma, cavernous hemangioma (n = 4), petroclival meningioma (n = 2), and clival chordoma. Temporary and permanent cranial nerve palsy as a procedure-related complication was seen in 11.8% (n = 2) each. Complete excision was achieved in 13 (n = 13/14) patients with tumors. Conclusion: FTDF unlocking and EDAC are elegant procedures providing reasonable access to the anterolateral skull base for myriad pathologies. Brain bulge, cavernous sinus bleeding, and losing the plane of dural duplication were significant challenges in switching from cadaveric to a clinical scenario.

Microneurosurgery for Spinal Dural Arteriovenous Fistula- Operative Video.

Background and Introduction: Spinal dural arteriovenous fistula (SDAVF) is a rare but curable condition. Microsurgery is a highly effective and readily affordable treatment modality. Objective: We present a surgical video of SDAVF to demonstrate the operative nuances involved. Surgical Technique: A 53-year-old wheelchair-bound man with spastic paraparesis for 1.5 years was found to have a SDAVF at L1/2 level with a single fistula point. During surgery, a L1-L2 laminectomy and durotomy revealed a dilated vein accompanying the nerve root exiting L1/2 foramen that showed early filling on indocyanine green (ICG) video angiography. This vein was occluded, and a segment of this vein was removed during surgery, which led to resumption of normal spinal cord perfusion. Results: The patient showed gradual recovery of lower limb motor power and improved to assisted ambulation after 3 months. Conclusions: Surgery is a simple, effective, and cost-effective treatment option in SDAVF.

Paraclinoid Segment Aneurysms of the Internal Carotid Artery: Surgical Clipping.

BACKGROUND: Paraclinoid segment aneurysms arise from the internal carotid artery (ICA) between the distal dural ring and the origin of the posterior communicating (PComm) artery. OBJECTIVE: This pictorial study presents videos showing clipping of paraclinoid segment aneurysms. MATERIALS AND METHODS: The various subtypes of these aneurysms, the nuances in the technique of clinoidectomy, and methods of proximal control are presented. RESULTS: Cavernous ICA is designated as C4, clinoidal segment (between the proximal and distal dural rings) as C5, and supraclinoid segment (between the distal dural ring up to the PComm artery as C6 segment. The techniques used for clipping various aneurysms are based upon their subtypes and location. In the first case, in a giant superior hypophyseal artery aneurysm directed toward the suprasellar region, an intradural clinoidectomy helped in accessing the neck of the aneurysm encroaching into the clinoidal segment of ICA. In the second case, concurrent bilateral "kissing" paraclinoid segment aneurysms were clipped using a unilateral approach. In the third case, clipping of a dissecting paraclinoid segment aneurysm is demonstrated. CONCLUSIONS: Surgery still represents the most definitive form of treatment. It may also be used to evacuate an intracerebral hematoma, with an extremely tortuous proximal vessel or in an aneurysm with complex anatomy, with blister aneurysms, or following aneurysm regrowth following a failed endovascular procedure. Assessing the three-dimensional anatomy of various segments of ICA is an important step.

Treading Toward Anterolateral Skull Base by Unlocking the Frontotemporal Dural Fold Along with Extradural Clinoidectomy: Translation from A Cadaver to Clinical Scenario.

BACKGROUND AND INTRODUCTION: Unlocking of the frontotemporal dural fold (FTDF) and extradural removal of the anterior clinoid process (EACP) are challenging but mandatory skills for micro-neurosurgeons. Despite the presence of an extensive body of literature on this subject, the translation of this cadaveric and 3D simulation to a real patient turns out to be a very demanding and difficult task. OBJECTIVE: This video is aimed to address the surgical nuances and major adjustments necessary in the unlocking of the FTDF and extradural ACP removal in an actual case for an early-career neurosurgeon. SURGICAL TECHNIQUE: A 40-year lady presented with features of acromegaly with radiological evidence of significant component of the tumor in the right cavernous sinus along with sellar suprasellar component. To achieve a good hormonal control, a complete tumor excision was required, which was achieved with FTDF and EACP removal. The cavernous sinus was approached through the Parkinson's triangle. RESULTS: The patient had uneventful recovery and good hormonal control at the 3-month follow-up. CONCLUSION: FTDF unlocking and EACP are elegant procedures and need to be learned by all neurosurgeons. This article will provide excellent teaching material for young neurosurgeons.

Modified Retromastoid Approach and Clipping of "High-Riding" VA-PICA Junction Aneurysm: An Operative Video.

BACKGROUND AND INTRODUCTION: Clipping an aneurysm on an elongated and tented V4 segment near the origin of the posterior inferior cerebellar artery (high-riding VA-PICA junction aneurysm) can be challenging. OBJECTIVE: We demonstrate the microsurgical clipping technique of such an aneurysm using a modified retromastoid approach (MRMA) and glossopharyngeal-cochlear triangle (GCT). SURGICAL TECHNIQUE: A 50-year-old female with a ruptured high-riding left VA-PICA junction aneurysm underwent an MRMA. Using segmental vessel isolation with proximal and distal temporary clips, this aneurysm was occluded through the GCT by applying a tandem clipping technique while preserving the PICA. RESULTS: The procedure was uneventful. Apart from transient ataxia, she recovered completely and maintains a good status at follow-up. CONCLUSION: In high-riding VA-PICA junction aneurysms, a conventional far lateral approach may create awkward viewing and working angles. An MRMA with a horizontal trajectory through the GCT may be a more appropriate strategy.

Spinal Dermoid and Epidermoid Cyst: An Institutional Experience and Clinical Insight into the Neural Tube Closure Models.

Objectives The spinal dermoid and epidermoid cysts (SDECs) are rare entities comprising less than 1% of pediatric intraspinal tumors. The present study aims to extrapolate the clinicoradiological data, in order to identify the most plausible neural tube closure model in human and provide a retrospective representation from our clinical experience. Materials and Methods We collected the details of all histologically proven, newly diagnosed primary SDECs who underwent excision over the past 20 years. Secondary or recurrent lesions and other spinal cord tumors were excluded. Surgical and follow-up details of these patients as well as those with associated spinal dysraphism were reviewed. Clinical and radiological follow-up revealed the recurrence in these inborn spinal cord disorders. Results A total of 73 patients were included retrospectively, having a mean age of 22.4 ± 13.3 years, and 41 (56.2%) cases fell in the first two decades of life. Twenty-four (32.9%) dermoid and 49 (67.1%) epidermoid cysts comprised the study population and 20 of them had associated spinal dysraphism. The distribution of SDECs was the most common in lumbosacral region ( n = 30) which was 10 times more common than in the sacral region ( n = 3). Bladder dysfunction 50 (68.5%) and pain 48 (65.7%) were the most common presenting complaints. During follow-up visits, 40/48 (83.3%) cases showed sensory improvement while 11/16 (68.7%) regained normal bowel function. There was no surgical mortality with recurrence seen in eight till the last follow-up. Conclusions The protracted clinical course of the spinal inclusion cysts mandates a long-term follow-up. The results of our study support the multisite closure model and attempt to provide a retrospective reflection of neural tube closure model in humans by using SDECs as the surrogate marker of neural tube closure defect.

Resistant Temporal Lobe Epilepsy: Initial Steps into a Bigger Epilepsy Surgery Program.

Background Among the patients of drug-resistant epilepsy, a subset which has focal impaired seizures localizes to the temporal lobe region (TLE). A majority of these cases are surgically amenable with anterior-medial temporal lobe resection or "lesionectomy." Objective In India, there is scarcity of "specialized centers" providing "comprehensive epilepsy care" and this dearth is further worse in populous states. In this article, we share our single center, observational, and retrospective experience of TLE in background of limited resources and utmost requirement. Methodology Our study is a retrospective analysis medically refractory epilepsy patients (2016-2019). Patients with medically refractory epilepsy were selected based upon our noninvasive protocol (clinical semiology, interictal scalp electroencephalography (EEG), long-term video EEG monitoring data, and magnetic resonance injury [MRI]). The follow-up was noted from the last out-patient visit record or through telephonic conversation (International League Against Epilepsy score). Results Of 23 cases of TLE ( n = 7, mesial temporal sclerosis; n = 16 temporal lobe like cavernomas, tumors, or arterio-venous malformations). Single photon emission computed tomography/positron emission tomography (SPECT/PET) was performed in five cases (three cases of ictal/interictal SPECT and two cases of PET scan) where there was discordance between EEG/clinical and MRI. The median follow-up was of 19 months with 18 cases being seizure free. Five cases were fully off the antiepileptic drug (AEDs) while in 15 cases, the AEDs dosages or the number were reduced. Average number of AEDs reduced from 2.9 in preoperative period to 1.2 postoperatively. Two cases had quadrantanopia and one case of cerebrospinal fluid leak. Conclusion A multidisciplinary and holistic approach is required for best patient care. The results of our initial surgical experience are encouraging.

A clinico-pathological and neuro-radiological study of angiomatous meningioma: Aggressive look with benign behaviour.

Angiomatous meningioma is a rare variant tumor classified as WHO grade 1 meningioma and accounts for about 2.1% of all meningioma. Their clinical presentation, surgical management, and prognosis are almost similar to the classical meningioma. Despite of benign nature and being comparatively small in size, they look aggressive on radiology images like massive peritumoral edema and intense contrast enhancement. Being a unique subtype of meningioma, the studies on angiomatous meningioma are very limited. In this cross sectional retrospective study, we described the clinical presentation, radiology, histopathological features and differential diagnosis of 30 cases of angiomatous meningioma from a single centre. The clinical parameters include demographic profile, symptoms and radiological findings including location, extent, pattern, histopathology with World Health Organization (WHO) grade-2016, extent of tumour excision, recurrence and surgical outcome. Incidence of angiomatous meningioma in our study was 2.46% with male predominance. The most common location in our study was convexity. 27 out of 30 had histopathology reports of angiomatous meningioma and 3 had lipoangiomatous meningioma. The high vascularity and disproportionate peri-tumoral edema makes it a surgical challenge for excision. The complications and surgical outcome were analysed. The radiological anticipation of tumour subtype, meticulous pre-operative planning and intraoperative precautions remains a key for success.

How Important Is to Know the Psychosocial Performance in an Operated Child of Meningioma? Devil Is in the Details.

BACKGROUND: Meningioma constitutes only 0.4%-4.1% of all the pediatric tumors. This article aims to find the impact of the pediatric meningioma surgery on the developmental and scholastic performance among these children over long-term follow-up. MATERIALS AND METHODS: This study is a retrospective analysis of all the histopathological proven pediatric meningioma and a cross-sectional analysis to study the functional outcome, using Malin's Intelligence Scale for Indian children (MISIC); scholastic performance was assessed from behavioral checklist for screening the learning disabled (BCSLD) and subjective self-filled questionnaire to know parent satisfaction. RESULTS: Twenty-eight patients (mean age 14.52 ± 0.722 years) (M:F = 16:12) were analyzed (6 [21.4%] were grade-schooler and 22 [78.5%] were teenage), with most common symptom being headache (n = 20, 71.4%) and mean duration of symptoms was 11.19 ± 16.25 days. The mean intelligent quotient (IQ) of grade-schooler was 83.4 ± 9.072 compared to 75.69 ± 9.903 among teenage group. The BCSLD analyses showed that the postoperative score was poorer. Similarly, an average change in BCSLD was observed among the patients with complications (10) compared to the patients without complication (8). CONCLUSION: The discontinuation in schooling or lack of alternative education may lead to poor MISIC scores, wrongly categorizing the children in poor IQ group. In pediatric benign disease such as meningioma, the holistic approach should be opted from the time of first visit to neurological care team.

Factors affecting seizure outcome in Long-term epilepsy associated tumors (LEATs) in children and young adolescents.

OBJECTIVE: Tumors with seizures as primary mode of presentation are collectively called Long-term epilepsy associated tumors (LEATs or Epileptomas). The overall survival is good so 'seizure outcome' becomes the primary goal rather than neuro-oncological outcome. METHODS: A retrospective analysis of our surgical database (2015-19) was done to find operated patients of intra-axial brain tumors with age less than 25-years and who had presented with seizures. RESULTS: The mean age at presentation was 16.44 years (SD + 6.82 years). Complex partial seizures/focal unaware seizures were the most common type of seizures encountered (n = 22) with mean duration of seizures was 49.50 months (SD + 31.04 months). The most common pathology was glioneuronal tumors (GNTs) (n = 17). Gross total resection (GTR) group had a significantly better seizure outcome as compared with the Subtotal resection (STR) group (p = 0.006). Presence of focal or partial seizure was a significant factor pointing towards a better seizure control (p = 0.005). CONCLUSION: The shorter duration of symptoms, partial/focal seizures and gross total excision were predictors of a good seizure-outcome. Age of the patient and the histopathology of the tumor does not affect seizure-outcome on comparing GNTs with non GNTs.

Peter Plus Syndrome: A Neurosurgeon's Perspective.

Peter plus syndrome (PPS) is a rare, hereditary (autosomal recessive) disorder characterized by a mutation in the beta-1,3-galactosyltransferase-like gene (chromosome 13q12), which causes impaired glycosylation of several structural and functional proteins throughout the body. Clinical signs and symptoms of PPS are highly variable and include structural malformations affecting multiple organ systems including central nervous system. We aim to discuss a neurosurgeon's perspective to PPS in this report. A 2-year-old boy presented with congenital dysmorphic facies, bilateral central corneal opacities, delayed developmental milestones, short-stature (75cm), rhizomelia with brachydactyly, and history of surgery for anal atresia on the second day of life. Screening craniospinal magnetic resonance imaging revealed mild ventriculomegaly, cavum septum pellucidum, cavum velum interpositum, vermian hypoplasia, and normal spine. Cytogenetic analysis showed a mutation in the beta-1,3-galactosyltransferase-like gene on chromosome 13. Clinical picture in our patient suggested the diagnosis of PPS. Parents often seek ophthalmological consultation due to visual impairment predominantly, and this syndrome largely remains unknown among neurosurgeons. Nonetheless, babies with PPS may present with neurological symptoms such as seizures, spastic diplegia, tinnitus, or hearing loss as well as a life-threatening neurosurgical emergency arising due to raised intracranial pressure. Therefore, the role of neurosurgeon becomes crucial in managing these cases.

Coexistent Cerebral Cavernous Malformation and Developmental Venous Anomaly: Does an Aggressive Natural History Always Call for Surgical Intervention?

Coexistence of cerebral cavernous malformations (CMs) and developmental venous anomaly (DVA) represents the most common form of mixed intracranial vascular malformations. Existing literature supports not only a possible causative role of DVA for de novo CMs but also a potentially detrimental effect on an associated CM, increasing the chances of hemorrhagic complications and growth in the latter. A 52-year-old gentleman presented to us with a 17-year long history of simple motor seizures on the left faciobrachial region. On magnetic resonance imaging (MRI) of the head, a 1.5 cm × 1.5 cm CM without any evidence of recent hemorrhage was identified in the left high frontal premotor area. There was a linear enhancement in the adjoining superior frontal sulcus on contrast MRI. On intra-arterial angiogram, this hyperintensity was confirmed to be a venous channel draining into the superior sagittal sinus. Thus, a diagnosis of cavernoma associated with a DVA was made. The patient was advised conservative treatment and he was doing well at follow-up. Unless diligently looked for, DVA associated with CM may be easily missed. The coexistence has pathophysiological and management implications. Despite the reported aggressive natural history, there is a scope for conservative treatment for these complex vascular malformations.