CAR T-cell therapy: a potential treatment strategy for pediatric midline gliomas.

Pediatric brain tumors are the primary cause of death in children with cancer. Diffuse midline glioma (DMG) and diffuse intrinsic pontine glioma (DIPG) are frequently unresectable due to their difficult access location, and 5-year survival remains less than 20%. Despite significant advances in tumor biology and genetics, treatment options remain limited and ineffective. Immunotherapy using T cells with a chimeric antigen receptor (CAR) that has been genetically engineered is quickly emerging as a new treatment option for these patients. High levels of expression were detected for both disialoganglioside (GD2) and B7-H3 in pediatric DMG/DIPG. Numerous studies have been conducted in recent years employing various generations of GD2-CAR T cells. The two most prevalent adverse effects found with this therapy are cytokine release syndrome, which varies in severity from mild constitutional symptoms to a high-grade disease associated with potentially fatal multi-organ failure, and neurotoxicity, known as CAR T-cell-related encephalopathy syndrome. During the acute phase of anticancer action, peri-tumoral neuro-inflammation might cause deadly hydrocephalus. The initial results of clinical trials show that the outcomes are not highly encouraging as B cell malignancies and myelomas. In vivo research on CAR T-cell therapy for DIPG has yielded encouraging results, but in human trials, the early results have shown potentially fatal side effects and very modest, but fleeting improvements. Solid tumors present a hindrance to CAR T-cell therapy because of the antigenic dilemma and the strong immune-suppressing tumor microenvironment.

Embracing Exoscope in the Arsenal of Spine Surgeons in the Low- and Middle-Income Countries.

BACKGROUND: Exoscope (EX) is a device that combines the convenience of an endoscope with the image clarity of an operating microscope (OM) to fill the void between the two. This study aims to compare the 2-dimensional EX with OM in spine surgeries and to explore its utility and feasibility in small and peripheral hospitals of low- and middle-income countries. METHODS: Eighty-two patients with intradural spinal tumors (extramedullary and intramedullary) aged more than 18 years were included between August 2021 and August 2023. Patients with other spinal pathologies were excluded. After each exoscopic surgery, the operating surgeon and assistant had to answer a questionnaire. Postoperatively, outcomes were measured as the length of hospital stay, cerebrospinal fluid leak, and number of reoperations. RESULTS: Thirty-seven patients were included in the OM group and 45 patients were included in the EX group. In 62%-67% of cases, the overall image quality of the EX was comparable to OM and in 29%-38% of cases, it was superior to the OM. The preparation and installation of the EX were much easier and better than the OM in 93%-100% of the cases. Maneuvering surgical instruments and workflow in the operating theater was much more convenient in the EX group (95%-100% of the cases). Ergonomics was far better in the EX group than in the OM group. CONCLUSIONS: Spine surgeons can embrace the benefits of EX and increase their range of surgeries to be performed at the small operation theater setup in low-middle income developing countries.

A rare case of occipital encephalocele presenting as the largest congenital head mass in an infant.

BACKGROUND: The prevalence of encephalocele is estimated to be 0.8-5.0 per 10,000 live births. The most frequent encephalocele is the occipital encephalocele. It is a congenital neural tube defect characterized by the protrusion or herniation of intracranial contents through a cranial defect. The term "giant/massive/large encephalocele" is used to describe an encephalocele that is significantly larger than the size of the head. CASE DESCRIPTION: A 2-month-old male infant presented in the neurosurgery outpatient department with one of the largest head masses over the posterior aspect since birth. The swelling was gradually progressive and developed ulceration over the swelling with intermittent cerebrospinal fluid (CSF) discharge but no associated weakness in limbs. Magnetic Resonance Imaging (MRI) brain showed a large occipital meningoencephalocele containing predominantly cyst with part of the cerebellar and occipital lobe. The surgery was planned. The sac contained CSF with the gliotic occipital lobe. The sac and gliotic brain tissue was excised. He had an uneventful postoperative course. CONCLUSION: Surgery serves several functions, including reducing the torque and weight of the head to allow for more normal motor development, removal of the thin, leaking scalp and dural closure to prevent CSF leak and subsequent infection, and improving the cosmetic and social issues that the child and family may have to endure.

Novel freehand frontal ventriculostomy in the supraorbital keyhole approach: technical note.

BACKGROUND AND OBJECTIVES: The narrow surgical passage provided by the supraorbital keyhole craniotomy restricts the instrument's maneuverability and presents a number of technical challenges. Inadequate brain relaxation may eventually result in unavoidable brain retraction and neurological impairments. The article aims to provide a novel intraoperative ventriculostomy to assist in overcoming the barrier of a narrow surgical corridor and assess its feasibility and safety compared to other techniques. METHODS: The craniometric point was planned on one normal computed tomography (CT) brain. The coordinates were verified on 43 magnetic resonance imaging (MRI) brain images uploaded on the neuronavigation system (StealthStation S8 version 1.0; Medtronic, Louisville, USA). The ventriculostomy point was 3 cm superior to glabella, 2 cm lateral to midline, 6 cm deep to the brain surface in the perpendicular direction, and parallel to the floor of the anterior cranial fossa. Subsequently, the abovementioned radiologically calculated ventriculostomy trajectory was confirmed on 32 consecutive patients (without neuronavigation) of suprasellar mass undergoing supraorbital craniotomy between February 2022 and April 2023. The technical issues, feasibility, and outcomes were assessed. RESULTS: Out of 32 patients, in 29 patients, ventricular hit was attained in a single attempt, and the rest 3 patients needed two attempts. The intraoperative ventricular hit rate was 100% with 90.6% success in a single attempt. No ventriculostomy-related complications occurred. Compared to ELD (external lumbar drainage), performing an intraoperative ventriculostomy had no discernible difference in the perception of the brain retraction force. Intraoperative ventriculostomy fully eliminated the low back pain or radiculopathy that patients with ELD rarely have even after drain removal. CONCLUSION: The novel intraoperative frontal ventriculostomy is a safe trajectory and is a valid alternative to Menovsky's ventriculostomy or external lumbar drainage. The authors recommend this technique be generally utilized in supraorbital keyhole approaches to optimize brain relaxation and minimize secondary adverse events.

Comparison of the Novel Two-Hand Technique Using the Saraj Endocath with the Traditional Three-Hand Technique in Transnasal Endoscopic Pituitary Surgery: An Innovation for the Future.

BACKGROUND: Endoscopic surgery has become the preferred treatment of sellar lesions because of its minimally invasive nature. Visualization is frequently obscured as a result of the frequent contact of the lens with blood and tissue debris. We seek to alleviate these problems and increase the efficiency and safety of neuroendoscopic surgery by introducing a new device (Saraj Endocath) combining the major function of suction with the endoscope. METHODS: A total of 75 patients with pituitary macroadenoma who were eligible for transnasal transsphenoidal endoscopic excision were included in the study between January 2020 and January 2023. Forty-one patients were operated on by the traditional 3-hand technique and 34 patients were operated on using the Saraj Endocath. The duration of surgery, in-out frequency of the endoscope, number of wipes needed to clean the endoscopic lens, and outcomes were assessed. A questionnaire regarding assistance, ergonomics, and instrument handling compared the Saraj Endocath procedure with the traditional technique. RESULTS: The 3-hand technique was converted into the 2-hand technique. The mean operative time using the novel technique with the Saraj Endocath was reduced significantly compared with the traditional technique. The mean number of in-out movements of the endoscope and the number of wipes per hour decreased significantly using the Saraj Endocath. There was no dependency on the assistant's expertise and skills. CONCLUSIONS: The use of the Saraj Endocath can effectively reduce the mean operative time by decreasing the frequency of in-out movement and wiping off the lens of the endoscope. It reduces fatigue, avoids clustering of instruments and hands, and minimizes the dependency on assistants.

A brief review of the monkeypox virus and emerging concerns for neuroinvasiveness.

Background: Amidst the ongoing COVID-19 pandemic, monkeypox virus (MPXV) disease has been recognized as another disease of pandemic nature by the World Health Organization. Nearly four decades after the eradication of smallpox, as half of the world population is naïve to ortho-pox viruses (supposedly due to lack of immunity by vaccination), MPXV remains the most pathogenic species of the family of poxviruses. Methods: The articles on MPXV were searched on PubMed/Medline and data were retrieved and analyzed. Results: Although reported as a disease of milder exanthem and lower mortality as compared to smallpox, the MPXV disease tends to be neuroinvasive. This article highlights the neurological signs and symptoms of MPXV disease and discusses, in brief, the management strategies. Conclusion: Neuroinvasive properties of the virus as demonstrated in in vitro studies, and further verified by neurological illnesses in patients, present a special threat to mankind. Clinicians must be prepared to recognize and treat these neurological complications and start treatment to limit long-lasting brain injury as seen in patients with COVID-19.

Posterior fossa giant adenoid cystic carcinoma with skull base invasion mimicking glomus jugulare: A case report and review of literature.

The author describes a rare case of giant adenoid cystic carcinoma (ACC) mimicking large paraganglioma with lower cranial nerve palsy. A 60-year-old female presented with a progressive increase in postauricular swelling with unilateral hearing loss, facial deviation, difficulty in swallowing, and hoarseness of voice. MRI brain showed highly vascular infiltrating and osteolytic mass suggestive of large glomus jugulare versus sarcoma. It was completely engulfing the jugular foramen and lower cranial nerves with bony erosion of the jugular foramen and occipital condyle. The whole mastoid was filled with the tumor. On digital subtraction angiography the majority of blood supply was from the occipital branch of the external carotid artery and vertebral artery. The patient underwent percutaneous embolization followed by external carotid ligation and resection of the mass. The postoperative course was uneventful. Histopathology was suggestive of mixed ACCs. The patient received radiotherapy. After 1 year of follow up no recurrence or distant metastasis was noted.

Management and outcome of unusual pediatric brain tumors: challenges experienced at a tertiary care center of a developing country.

PURPOSE: Primary brain tumors are the most frequent solid pediatric tumors, accounting for 40-50% of all cancers in children. Eighty to ninety percent of the 250,000 new cases of pediatric cancer each year are discovered in low and middle-income nations, where nearly 88 percent of the world's children reside. This article aims to emphasize the unusual presentation, management, and surgical outcome of complex pediatric brain tumors. METHODS: We performed a retrospective study of patients who were admitted to the neurosurgery department with unusual pediatric brain tumors between March 1, 2019, and March 1, 2022. The study included pediatric patients up to age 18 years. We included those pediatric brain tumors whose (i) location was uncommon, or (ii) presented with unusual clinical presentation, or (iii) histopathology suggested to be a rare tumor, or (iv) radiological features were atypical. RESULTS: We included 9 cases of rare unusual pediatric brain tumors. Three out of 9 cases required preoperative embolization due to its hypervascular nature on digital subtraction angiography (DSA). All patients underwent surgical excision within 24-48 h of tumor devascularization. One out of 9 cases died in follow-up period due to pleural effusion and distant metastasis to lungs. CONCLUSION: Treatment considerations for unusual pediatric brain tumors include a comprehensive multidisciplinary approach, including community-based screening and proper referral system for early treatment, a variety of treatment modalities, and sophisticated follow-up strategy. Government shall work in coherence with tertiary centers to spread social awareness and provide various financial scheme to prevent treatment dropouts.

Prognostic Factors and Outcome of Surgically Treated Supratentorial versus Infratentorial Epidural Hematoma in Pediatrics: A Comparative Study of 350 Patients at a Tertiary Center of a Developing Country.

PURPOSE: In children, epidural hematomas (EDHs) constitute around 2% to 3% of all head traumas. The aim of this study is to compare the manifestation, prognostic factors, and outcome of surgically treated supratentorial with infratentorial EDHs in pediatric patients. METHODS: This is a hospital-based single-center, retrospective study of 350 pediatric patients admitted between January 2016 and December 2021. All pediatric patients to 18 years of age with posttraumatic EDHs with or without other intracranial/extracranial injuries who underwent surgical evacuation were included in the study. Posttraumatic EDHs treated conservatively during the hospital stay and any EDH unrelated to head trauma were excluded. Glasgow Outcome Scale (GOS) score was used to assess functional outcomes at discharge. The status of the patients at 3-month follow-up was assessed by using the pediatric version of the Glasgow Outcome Scale-Extended (GOS-E Peds) Score. RESULTS: Out of 350 patients, 310 had supratentorial EDH and 40 had infratentorial EDH. In supratentorial EDH, the volume of hematoma, mass effect, and the time interval between trauma and surgery correlated with functional outcome (GOS) at discharge. Anisocoria, hypotension, and intradural injury were associated with functional as well as behavioral outcomes (GOS-E Peds) in the supratentorial EDH group. The severity of the injury was correlated with the functional and behavioral outcomes in both groups. CONCLUSIONS: Infratentorial EDH has better clinical outcomes than supratentorial EDH in surgically treated pediatric patients. The most significant and consistent factor influencing the outcome in both groups was the Glasgow Coma Score on admission.

COVID-19-induced atypical pneumonia in an unseparated adult conjoined twin in a remote setup of developing country: management by telemedicine. Illustrative case.

BACKGROUND: Telemedicine is the use of wireless communications and Web-based technologies to provide healthcare and services. The coronavirus disease 2019 (COVID-19) pandemic has posed an unprecedented challenge to healthcare providers, who have been forced to change their usual mode of service delivery and have been given an opportunity to adopt the concept of telemedicine. OBSERVATIONS: A 40-year-old underprivileged and unseparated conjoined twin (pygopagus) had dorsal spine tuberculosis and was on a regular follow-up through telemedicine due to the ongoing COVID-19 pandemic and embarrassment they had to face in public appearances. After a few months, they contracted COVID-19 infection after which they were shifted to our tertiary care center. Several unique challenges were encountered during the 4-day course of management. Ultimately, they died and left us with questions of whether we are really prepared to tackle these challenges. LESSONS: The general public should be made aware of such groups of patients and to encourage them to follow COVID-appropriate behavior. Vaccinations should be given on a priority basis to these subsets. A more robust approach of telemedicine consultation is required for management of patients in remote areas at the time of a pandemic. Ventilator management of these patients is still intriguing.

Filar arteriovenous fistula mimicking upper motor neuron palsy: A case report with review of the literature.

Background: Filar A-V fistula is a rare entity. It requires a high degree of suspicion to diagnose. Magnetic resonance imaging (MRI) findings are often nonspecific and spinal angiogram is required to diagnose it. Case Description: A 63-year-old male patient presented with Grade 4 spastic paraplegia and significant sensory disturbance below D8 level along with severe vesicorectal dysfunction. On imaging flow voids were present at lower dorsal and lumbar level in MRI (T2 sequence). Patient underwent spinal digital substraction angiography (DSA) which was suggestive of filar fistula at L4-L5 level. Patient underwent surgical exploration with L4-5 laminectomy. Feeding artery was identified using indigocyanine green (ICG) dye and excised along with filum and dilated vessels. Patient recovered symptomatically in postoperative period. Conclusion: Filar fistula is a rare lesion and it presents with long standing progressive congestive myelopathy. It requires a high degree of suspicion to diagnose it. DSA is the gold standard for diagnosis and management planning. Surgical approach utilizing the ICG dye is best treatment options in such cases.

Surgical management of post-COVID invasive rhino-orbito-cerebral mucormycosis and its outcomes: Role of neurosurgeons in a tertiary care center.

Background: Mucormycosis, which was previously considered to be rare, has emerged with a new challenge in patients infected with or recovering from COVID-19. Immunocompromised patients are particularly prone to developing this disease. The most common form of presentation is rhino-orbito-cerebral mucormycosis (ROCM). We present various neurosurgical approaches to an entire spectrum of its clinical manifestations. Methods: This is a retrospective study of patients who were admitted to the neurosurgery department with ROCM and a history of COVID-19 infection between November 1, 2020, and September 1, 2021. All cases of ROCM underwent contrast-enhanced computed tomography/magnetic resonance imaging of the brain, paranasal sinuses, and orbit. A tissue biopsy was sent for histopathological analysis. All confirmed cases received liposomal amphotericin B and surgical treatment was immediately undertaken. Results: Out of 200 patients with ROCM, 40 patients presented with neurological manifestations. Seven out of 40 patients had focal lesions in the brain and skull bone that needed neurosurgical intervention along with sinus debridement and antifungal treatment. These seven patients presented with different clinical manifestations: large-vessel stroke (one), medium-vessel stroke (one), frontal lobe abscess (one), frontal bone osteomyelitis (two), isolated central nervous system involvement (one), and mucor mimicking trigeminal schwannoma (one). The surgical intervention included decompressive craniectomy, frontal craniotomy, subtemporal craniotomy, and a minimally invasive supraorbital keyhole approach. Conclusion: In high-risk patients, a high level of clinical suspicion combined with appropriate investigations should be performed as soon as possible. Symptoms and early warning signs should not be overlooked, as treatment delays can be fatal. A minimally invasive surgical approach is possible in view of decreasing the morbidity of large craniotomy.

High-definition two-dimension video telescope operating monitor-assisted brain and spinal surgery in pediatrics: is it an acceptable substitute for microscopic surgery?

PURPOSE: Vision and ergonomics are crucial variables for successful outcomes during neurosurgery procedures. Two-dimension video telescope operating monitor (VITOM) exoscope has emerged as an alternative, which is cheaper than microscope. The aim of this study is to evaluate the clinical utility of 2D VITOM and to compare its merits and demerits with respect to microscope. METHODS: VITOM 2D (Karl Storz, Germany) was used in 9 cranial and 5 spinal pediatric cases. While KINEVO operative microscope (Carl Zeiss, Germany) was used in 12 cranial and 6 spinal pediatric patients. All surgeries were performed by single senior neurosurgeon. The author's experience and opinions, as well as qualitative data, were analyzed. A comparison was made on image quality, illumination, field of view, and magnification of the operative field and ergonomics. RESULTS: Seven out of 9 cranial pediatric cases were switched from VITOM 2D to operative microscope due to low-image definition in depth of cranial cavity. Poor visualization of bleeding source in surgical field was another major drawback. Two cranial cases in which exoscope were used exclusively, included superficial tumors. In all 5 spinal cases, VITOM 2D was successfully used without any major difficulty. The exoscope's advantages were observed in ergonomics and ease in switching to naked eyes, but the microscope's field of view, illumination, magnification, and user-friendliness was considered superior. CONCLUSION: 2D-VITOM is best suited for spinal and superficial cranial tumors. However, a lot of modifications are to be done especially in optics to become a substitute for operative microscope.

Awareness of an Primitive Art for Treating Otological Diseases in Present Covid-19 Scenario: Need of the Hour.

In current scenario of Covid-19 pandemic spread of virus via aerosol generating procedures has become a special concern in otorhinolaryngology community. Motive of this study is to spread awareness of an ancient forgotten method of performing mastoidectomy through which risk of virus infection can be greatly reduced among otologic surgeons. Retrospective Observational study. Three patients of chronic otitis media with complication were operatively intervened with combined approach of otorhinolaryngology and neurosurgeons. Mastoidectomies were performed without drilling. Instruments used were small, medium and large size gouge, chisel and hammer, curettes, kerrison punches and other microscopic ear instruments. All three patients recovered well without any residual disease or cavity problems. Referring ancient practices like above in this COVID era may prove an important tool in addressing surgical urgencies while combating transmission risks at the same time. One should be vigilant and versatile in surgical techniques in order to serve the needy and save the saviours simultaneously.

Comparative analysis of the risk factors influencing recovery of function from Oculomotor nerve Palsy in unruptured and ruptured Posterior Communicating Artery Aneurysms.

AIM: Few studies have analyzed and compared the risk factors responsible for the recovery of function from oculomotor nerve palsy (OMNP) between cases with unruptured and ruptured posterior communicating artery (PCOM) aneurysms. We also have assessed these risk factors and simultaneously compared the benefits of procedures (clipping vs. coiling) in the recovery of function from OMNP. MATERIAL AND METHODS: Among the 225 cases of aneurysm treated in our department between July 2018 and February 2020, 25 patients with PCOM aneurysm with OMNP (unruptured: n = 13; ruptured: n = 12) were retrospectively analysed . RESULTS: The average duration from onset of symptoms to treatment in unruptured PCOM aneurysm cases was 13.33 ± 3.76 days compared with 7.41 ± 2.42 days in ruptured aneurysm cases. Moreover, an 80% improvement was observed when OMNP was treated within 17 days with the earliest improvement noticed in 33.05 ± 18.75 days in unruptured aneurysm cases compared with 39.66 ± 31.75 days in ruptured PCOM aneurysm cases. Stepwise logistic regression analysis revealed that the type of aneurysm (better recovery in unruptured aneurysm cases) was a significant risk factor (p = 0.0126), but not the procedure (clipping vs. coiling) performed, for function recovery from OMNP. CONCLUSION: Patients with unruptured PCOM aneurysms with OMNP have a better recovery rate than those with ruptured PCOM aneurysms. No procedural (clipping vs. coiling) advantages were observed on the recovery of function from OMNP. Transmitted pulsation reduction significantly affects the recovery of function from OMNP.

Analysis of Giant Intraventricular and Extraventricular Epidermoids, Defining Risk Factors for Recurrence, an Institutional Experience.

BACKGROUND: Multicompartmental intraventricular epidermoids behave differently from multicompartmental extraventricular lesions and localized lesions during its management. Few studies are available which have analyzed risk factors separately in these groups of cases for recurrence of these lesions and time to recur. MATERIALS AND METHODS: In this retrospective observational study, 72 cases of intracranial epidermoid were treated over a span of 7 years. Cases were categorized into three groups. Group 1 comprised 15% (11/72) of cases with intraventricular multicompartmental, Group 2 with 22% (16/72) extraventricular giant tumors with multicompartmental involvement and size >4.5 cm, and Group 3 comprised 63% (45/72) of patients with lesions <4.5 cm and localized. Data pertaining to demography, clinical and radiological features, surgery performed, postoperative complication, histology, and follow-up were obtained from medical records available in the institute. RESULTS: The average duration to treat was 1.86 ± 0.52 (standard deviation [SD]) years, with headache as a major complaint in all the groups. Combined endoscope-assisted microsurgery was performed in 38.8% (28/72), microsurgery in 54.1% (39/72), and endoscopic excision in 6.9% (5/72) of cases. Tumor calcification was found in 23.6% (17/72) and preoperative capsular enhancement was seen in 19.4% (14/72) which persisted in 79% (11/14) of cases postoperatively on subsequent follow-up suggesting recurrence. On stepwise logistic regression analysis, preoperative capsular enhancement was a strong predictor of recurrence of tumor (P = 0.001). The average follow-up was 46 ± 14.92 (SD) months in Group 1, 52.34 ± 11.45 (SD) months in Group 2, and 63.36 ± 18.42 (SD) months in Group 3. CONCLUSION: Although the intracranial epidermoid is known to recur after long interval, tumor with specific characteristics can recur in short span of 5-6 years. Tumor characteristics such as preoperative capsular enhancement, multicompartmental distribution in vertebrobasilar territory, large size, and presence of calcification are strong predictors for recurrence. Performing endoscope-assisted microsurgery can decrease the postoperative morbidities but does not reduce the recurrence risk.