Calvarial Lesions: A Tertiary Centre's Experience Over Fifteen Years.

BACKGROUND: The human calvaria harbors a variety of pathology and majority of them are incidentally noticed as painless swelling. The aim of the present study is to describe the histopathological subtypes of calvarial lesions, their management and factors affecting their surgical outcome at a tertiary care referral center. MATERIAL AND METHODS: All patients who underwent excision of the calvarial lesions over the last 15 years (from January 2005 to July 2019) were included in this study. Patients having calvarial pathology of infective origin and recurrent lesions were excluded. Any patient with multiple calvarial lesions who have been operated more than one time for same histopathological diagnosis was counted as one patient. We studied Karnofsky Performance Status (KPS) scores and radiological changes at 3-month follow up. RESULTS: Total 65 patients were recruited in this retrospective observational study. The median age of patients in the study was 29 years (range: 8 years to 68 years). Fibrous dysplasia 20 (30.7%) was the commonest lesion while metastatic thyroid carcinoma 3 (4.6%) was the most common malignant pathology. Complete excision was performed in 51 (78.5%) of patients while in 14 (21.5%) cases, subtotal or near total decompression were achieved. After three months of surgery, there was significant improvement in the KPS score (P < 0.00001). Duration of follow up ranges from 6 months to 5 years with 4 mortality in the study. CONCLUSIONS: Most of the calvarial tumors were benign and surgically addressable. The malignant lesions were scattered with diverse underlying pathology and required individualized holistic approach.

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.

Surgery in High-Grade Insular Tumors: Oncological and Seizure Outcomes from 41 Consecutive Patients.

BACKGROUND: Insular high-grade gliomas are uncommon and constitute approximately 10% of all intracranial high-grade gliomas. Several publications in the recent years have thrown substantial light in the understanding of insular low-grade gliomas. However, there is a paucity of information concerning the spectrum of high-grade lesions affecting the insula, the mode of presentation vis-à-vis low-grade gliomas, and the survival rates to modern therapy. AIMS AND OBJECTIVES: We aim to highlight various clinical patterns, histo-pathological spectrum and the survival rates in patients with high-grade insular lesions. Also, we explore the factors that govern favourable outcomes. MATERIALS AND METHODS: A retrospective study of 41 patients operated for high-grade insular tumors at our institute between March 2010 to December 2018 was done to evaluate the clinico-radiological features, surgical nuances, survival rates and seizure outcomes. RESULTS: Raised intracranial pressure was the most frequent clinical presentation (n=28/41, 68.3%). Nearly 60% of the patients (n=25) had involvement of all four Berger-Sanai zones. The high-grade tumors encountered in our series were: glioblastoma (n=15), gliosarcoma (n=3), and embryonal tumor, not otherwise specified in 3 patients, while 21 patients had grade 3 astrocytoma. 33 out of 41 patients (80.5%) in our study showed excellent seizure control (ILAE grade 1A) at follow-up. Clinical presentation with seizures (P = 0.01, HR=0.3), WHO grade IV histopathology (P = 0.04, HR=3.7) and development of recurrence (P = 0.05, HR=5.5) were found to be independent predictors of OS. CONCLUSION: Insular high-grade gliomas are commoner than thought and nearly half of these are grade IV tumors (51%). A presentation with seizures may indicate precursor low-grade gliomas and portend a better survival. A maximum "safe" surgical resection, keeping the postoperative quality of life in mind, should be the goal.

A Single-Center Surgical Experience of Interhemispheric Epidermoids and Proposal of a New Radiological Classification.

BACKGROUND: Epidermoids account for <1.5% of all intracranial tumors. Overall, the cerebellopontine angle has been the most commonly reported location. In the supratentorial compartment, epidermoids will usually be found in the suprasellar area, sylvian fissure, and intraventricular area. The interhemispheric fissure represents an extremely rare location for supratentorial epidermoids, with limited cases reported previously. Surgery of an interhemispheric epidermoid can be challenging because of its adherence to the anterior cerebral artery branches or the brain parenchyma itself. In the present study, we have reported the largest Series of interhemispheric epidermoid tumors to better understand the clinical behavior, radiological features, and surgical outcomes. METHODS: In the present study, we retrospectively evaluated the data from 22 consecutive patients with interhemispheric epidermoids treated surgically during the past 10 years (2009-2019) at our center. During the same study period, we had treated 2355 patients with brain tumors (both primary and secondary), including 262 patients with intracranial epidermoids. Therefore, interhemispheric epidermoids comprised 0.009% of all brain tumors and 8.4% of all intracranial epidermoids in our experience. These patients all had newly diagnosed tumors and had undergone surgery for the first time at our center. All surgical specimens had been confirmed histopathologically. RESULTS: The mean age of the patients was 31 years (range, 17-61 years), with a male predilection. Seizures and headache were the most common mode of presentation. The average tumor size was 5.2 cm (range, 3.5-10 cm). Of the 22 tumors, 21 were in the anterior two thirds of the fissure. Anatomical distortion of the corpus callosum was seen in 13 patients (59%). The extent of excision was total in 17 (77.3%), near total in 3 (13.6%), and subtotal in 2 (9%) patients. The near or subtotal excisions had been necessary because of either tumor adherence to the pericallosal artery (n = 3) or a missed tumor lobule (n = 2). The mean follow-up time was 32 months, with no tumor recurrence. CONCLUSION: Interhemispheric epidermoids can often reach a large size with substantial lateral extension found at diagnosis. The position of the anterior cerebral Artery branches can help to differentiate interhemispheric epidermoids from radiologically similar corpus callosum, velum interpositum, and cavum septum pellucidum epidermoids. Complete surgical excision with minimal complications is feasible, with good long-term outcomes.

Endoscopic Third Ventriculostomy and Simultaneous Tumor Biopsy in Pineal Region Tumors using the "Single Burr Hole" Technique: An Analysis of 34 Cases.

BACKGROUND: Pineal region tumors often present with hydrocephalus. Endoscopic third ventriculostomy (ETV) and simultaneous tumor biopsy remain a minimally invasive procedure offering both diagnostic and therapeutic advantages in the management of these tumors. However, different operative techniques have been described in the literature. AIM: The aim is to study the ETV success rate, diagnostic rate of simultaneous tumor biopsy, complications, and follow-up of patients of pineal region tumors managed with ETV and simultaneous tumor biopsy using the single burr hole technique. METHODS: The study was performed by retrospectively reviewing the records of patients of pineal region tumors managed by simultaneous ETV and tumor biopsy using a "single burr hole" technique from January 2012 to December 2019. RESULTS: Thirty-four patients (22 males and 12 females) with a mean age of 28.7 years were analyzed. ETV was successful in relieving hydrocephalus in 29 (87.8%) patients. Three patients needed a ventriculoperitoneal shunt, and one required Ommaya reservoir placement for persistent hydrocephalus. Histological diagnosis was successfully established in 26 (78.8%) patients. There were two procedure-related mortalities. Two patients underwent craniotomy and tumor excision subsequently. Radiotherapy was given to 11 patients, and 9 patients were managed by observation alone. The mean follow-up of our study was 15.8 months. CONCLUSIONS: Simultaneous ETV and tumor biopsy using a single burr hole technique is a safe, minimally invasive procedure for the management of pineal region tumors.

Intradural Extramedullary Nonconus Nonfilum Spinal Ependymomas: Report of a Rare Variant and Newer Insights into Their Histogenesis with Proposal of a Classification Scheme and a Management Algorithm Based on a Review of Literature.

BACKGROUND: Ependymomas are common intramedullary spinal tumors but there are scattered reports of this tumor presenting with exophytic growth patterns masquerading as intradural extramedullary (IDEM) tumors. Such IDEM ependymomas are seldom suspected preoperatively and it is only during surgery that their existence is revealed. Little is known of such rare growth patterns of an otherwise common intramedullary spinal cord tumor, their characteristics, and their management considerations. METHODS: We present a case of an exophytic dorsal ependymoma with a stalk like attachment to the spinal cord and the surgical management in a 24-year old woman. An extensive literature search was carried out on all prominent databases to find out similar cases reported earlier. We excluded filum/conus ependymoma, purely extradural spinal ependymoma as well as the extraspinal ependymomas. Details of each case reported before were obtained and tabulated. RESULTS: IDEM ependymomas have been reported in 54 patients so far, including the present case. Four patterns of growth emerged from the literature review: intramedullary ependymoma with exophytic component (group I, n = 9), exophytic IDEM ependymoma without intramedullary component (group II, n = 6), IDEM ependymoma arising from nerve roots (group III, n = 7), and pure IDEM ependymoma (group IV, n = 32). Except in group I, IDEM ependymoma affects females more frequently, without any specific age predilection. Thoracic spinal cord/canal is the most common location across all groups. Multifocal disease, craniospinal dissemination, and recurrences tend to be maximum in group IV. CONCLUSIONS: IDEM ependymomas are more common in thoracic segment of the cord and broadly divisible into 4 subgroups. We suggest a subpial origin of group II IDEM ependymomas. We also advocate proliferation index estimation in grade II ependymomas to enable formulation of an optimal management plan.

"Contralateral Dry Eye in Hemifacial Spasm:" A New Clinical Sign.

Hemifacial spasm (HFS) is a benign condition characterized by unilateral, involuntary, paroxysmal clonic and tonic contractions of the facial muscles. This condition usually results from a focal demyelination at the root entry zone of the 7th cranial nerve secondary to a vascular loop compression, and hence, it responds to microvascular decompression (MVD) surgery, similar to trigeminal neuralgia. Herein, we report an interesting clinical finding of a contralateral dry eye in the case of HFS, which has not been described previously and discuss the possible underlying mechanisms. A 53-year-old man presented with a 6-month history of involuntary twitching movements of the left hemiface that persisted during sleep, consistent with the diagnosis of HFS. The patient's attempts to voluntarily control the troublesome involuntary left-sided eye blinking led to the development of dryness and reddening of the right eye. Corneal reflex, both direct and consensual, was intact bilaterally, and an ophthalmological examination ruled out the diagnosis of conjunctivitis. The patient underwent MVD of the facial nerve. To our surprise and validating our supposition, his contralateral conjunctival hyperemia and dry eye resolved within a day of surgery, along with complete resolution of the HFS. HFS can lead to the contralateral dry eye from the voluntary suppression of ocular blinking which resolves following MVD. We demonstrate this finding for the first time and believe its recognition may be of value in the patient management.

Isolated Intraventricular Chronic Mucormycosis in an Immunocompetent Infant: A Rare Case with Review of the Literature.

BACKGROUND: Mucormycosis of the central nervous system is an uncommon infection caused by saprophytic or parasitic fungi of the subphylum Mucormycotina and order Mucorales viz. Rhizopus, Mucor, and Rhizomucor. Isolated, chronic involvement of the central nervous system is a rare occurrence. To the best of our knowledge, isolated chronic ventricular involvement in an infant has not been reported previously. Isolated intracerebral mucormycosis is a disease of the immunocompromised patient, and to date only 6 cases have been reported in immunocompetent patients, including 2 pediatric cases. CASE DESCRIPTION: We present the case of an immunocompetent infant presenting with features of increased intracranial tension. He underwent cerebrospinal fluid diversion and was found to harbor mucormycosis on histopathologic examination of intraventricular debris. We also present a brief review of the relevant literature. CONCLUSIONS: Although mucormycosis is an acute fulminant infection, chronic isolated cerebral cases are known in the immunocompetent patient. Patients also may present with isolated hydrocephalus, and hence fungal infection must be ruled out in all, especially if a shunt is warranted.

Symptomatic extradural spinal arachnoid cyst: More than a simple herniated sac.

INTRODUCTION AND STUDY DESIGN: Spinal arachnoid cyst is an uncommon entity, presenting with radiculopathy or paraparesis. These cysts are usually found in intradural extramedullary region; and the extradural region is a rare location. The exact pathogenesis in the existence of these cysts in the extradural region is still debated. In this article, we have retrospectively analyzed the clinical profile of the rare extradural arachnoid cyst (EDAC). METHODOLOGY: In this study, 19 patients of EDAC operated at our institute between January 2006 to June 2016 are analyzed. All patients with the Oswestry disability index score of >20 were managed surgically (open laminectomy and cyst excision). The clinical outcome was assessed at using 5-point satisfaction scale and McCormick grading. RESULTS: All 13 operated patients had EDACs with communication with the intradural compartment. In 11 (84.6%) patients, cyst wall was excised completely, in 2 (15.3%) patients underwent partial excision of cyst wall; dural communication was closed in all patients (n = 13). None of the patients had clinical deterioration or radiological recurrence till the last follow-up. Mean follow-up was 52.2 months (range 1-160); all patients were satisfied after surgery (median score was 3). DISCUSSION: Symptomatic EDACs account <2% of all spinal tumors. The EDACs have communication with the intradural compartment. In our article, we have discussed the approach and management of EDAC, including minimally invasive percutaneous procedures. CONCLUSION: One should aim for preoperative or intraoperative localization of dural communication and try to disconnect the extradural cyst from the intradural connection to prevent recurrence. Surgical treatment is complete excision of the cyst.