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Background: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection., Methods: A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described., Results: The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired., Conclusion: IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Non-chordomatous bony tumors of the clivus are extremely rare. Site, extent, and aggressiveness of tumor limits the extent of resection. It poses challenge to the neurosurgeons due to the complexity of anatomy. There is paucity of literature exclusively on non-chordomatous bone tumors of the clivus in young adults., Objectives: To analyze the clinical presentation, imaging findings, surgical approach, complications, and outcome of primary clival bony tumors in young adults., Methods: We retrospectively reviewed children and young adults with primary clival bony tumors excluding chordoma who underwent surgical resection between years 2010 and 2023 in our center. We analyzed the demographic details, imaging findings, operative variables, perioperative complications, length of stay, complications, and outcome at latest follow-up., Results: The mean age was 17.5 ± 1.73 years (range 16 to 19 years). Headache was the presenting complaint in all four patients (100%). The mean duration of symptom was 7.25 ± 3.2 months (range 5 to 12 months). The tumor was localized in clivus in all four patients (100%). The mean length of stay in hospital was 30.5 ± 13.48 days (range 11 to 40 days). All patients underwent surgical treatment. Surgical approaches used were anterior approach in four patients (100%). Gross total excision was performed in one patient (25%), sub-total excision was performed in two patients (50%), and tumor decompression was performed in one patient (25%). Of these, three were designated as having benign tumors and one had a malignant tumor. There was no perioperative mortality. There was one mortality (25%) on 2 months follow-up due to tumor progression. Three patients (75%) had improved symptomatically at latest follow-up. Two patients (50%) received adjuvant chemoradiotherapy. The mean follow-up was 38 ± 39.29 months (range 2 to 72 months)., Conclusion: Non-chordomatous bony tumors of the clivus are rare and often underestimated. Surgery is the treatment of choice. Tumor consistency and adhesion to critical neurovascular structures precludes gross total resection. Various approaches are in the armamentarium. Approach to be decided based on the expertise of the neurosurgeon to achieve safe maximal resection. Multidisciplinary approach is mandatory for streamlined management. Adjuvant therapy is decided based on the residual tumor following surgery., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Epilepsy surgery for pediatric drug-resistant epilepsy has been shown to improve seizure control, enhance patient and family QoL, and reduce mortality. However, diagnostic tools and surgical capacity are less accessible worldwide. The International Society Pediatric Neurosurgery (ISPN) has established a Pediatric Epilepsy Surgery Interest Group (PESIG), aiming to enhance global collaboration in research and educational aspects. The goals of this manuscript are to introduce PESIG and analyze geographical differences of epilepsy surgery and technology availability., Methods: PESIG was established (2022) following an ISPN executive board decision. Using a standardized form, we surveyed the PESIG members, collecting and analyzing data regarding geographical distribution, and availability of various epilepsy treatment-related technologies., Results: Two hundred eighty-two members registered in PESIG from 70 countries, over 6 continents, were included. We categorized the countries by GDP as follows: low, lower-medium, upper-medium, and high income. The most commonly available technology was vagus nerve stimulation 68%. Stereoelectroencephalography was available for 58%. North America had statistically significant greater availability compared to other continents. Europe had greater availability compared to Africa, Asia, and South (Latin) America. Asia had greater availability compared to Africa. High-income countries had statistically significant greater availability compared to other income groups; there was no significant difference between the other income-level subgroups., Conclusion: There is a clear discrepancy between countries and continents regarding access to epilepsy surgery technologies. This strengthens the need for collaboration between neurologists and neurosurgeons from around the world, to enhance medical education and training, as well as to increase technological availability., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Spinal deformities are common in Marfan syndrome (MFS). They usually involve the thoraco-lumbar spine but rarely involves the cervical spine. Kyphosis is the common spine deformity of the cervical spine and mandates surgical correction as they are at risk of neurological deterioration since they are refractory to conservative management. Few studies of surgical correction of spine deformity included cervical deformity., Objectives: To analyze the challenges faced during surgery, clinical and radiological outcome, and complications following surgical correction for cervical kyphosis in Marfan syndrome., Methods: We identified that 5 patients with a diagnosis of MFS with cervical kyphosis who underwent fusion surgery between the years 2010 and 2022 were reviewed, retrospectively. We analyzed the demographic details, radiological parameters, operative variables (blood loss and nuances), perioperative complications, length of stay, clinical and radiological outcome, and complications following fusion surgery for cervical kyphosis in MFS., Results: The mean age of patients was 16.6 ± 4.72 years (range, 12-23 years). The average kyphotic vertebra involved is 3 ± 0.7 bodies (range 2-4) with 2 patients with thoracic deformity. All patients underwent surgical deformity correction. All patients improved clinically with Nurick grade (pre vs. post: 3.4 vs. 2.2) and mJOA (pre vs. post: 8.2 vs. 12.6). There was significant deformity correction from 37.48° to 9.1°. Mean blood loss encountered was 900 ± 173.2 ml. Perioperative complications: wound complication with CSF leak (1). Late complications: ventilator dependence (1) and junctional kyphosis (1). Mean length of hospital stay was 103 ± 178.9 days. All patients were doing symptomatically better after mean follow-up of 58 ± 28.32 months. One patient is bedridden and hospitalized., Conclusion: Cervical kyphosis is a rare spine deformity in patients with MFS, and they usually present with neurological deterioration mandating surgical correction. Multidisciplinary approach (pediatrics, genetics and cardiology) is required for systematic evaluation of these patients. They should be evaluated with necessary imaging to rule out associated spinal deformity (atlanto-axial subluxation, scoliosis, and intraspinal pathology like ductal ectasia). Our results suggest better surgical outcome in terms of low operative complications with neurologic improvement in MFS patients. These patients require regular follow-up to identify late complications (instrument failure, non-union, and pseudarthrosis)., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Intracranial myeloid sarcoma is a rare extramedullary presentation of acute myeloid leukemia (AML). It can involve the meninges and ependyma presenting as extra-axial mass lesion. Rarely, it can also invade the brain parenchyma. It is commonly seen in children. It is usually misdiagnosed due to its close resemblance to other intracranial tumors (meningioma, metastasis, Ewing's sarcomas, and lymphoma). These are underdiagnosed if they precede the diagnosis of leukemia., Case Report: A 7-year-old boy with isolated intracranial myeloid sarcoma who presented with raised intracranial pressure (ICP) which was successfully managed by surgical excision., Conclusion: Isolated intracranial myeloid sarcoma is a rare presentation of AML. Leukemia can be diagnosed early during the postoperative period and can be started on therapy timely. These patients requires regular follow-ups (clinical, laboratory and radiological) to detect relapses early., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To study clinical and radiological outcomes of pediatric cervical kyphosis correction with a standalone posterior cervical approach. Cervical spine kyphotic deformity in pediatric age group is a distinct entity and the management is challenging. Pediatric cervical kyphosis is less often encountered, and literature is sparse with only few case series. Management algorithms are devised keeping the flexibility of the deformity at the core of decision making. Circumferential fusion is mostly recommended for non-flexible (rigid) kyphosis., Methods: Authors present a single center retrospective analysis of cases of pediatric cervical kyphosis managed by a standalone posterior approach. Pre- and post-operative clinical and radiological parameters were recorded and analyzed. Changes in neurological status, kyphosis correction and bony fusion were assessed. Surgical and implant related complications were noted., Results: Seven cases (6 male, 1 female) were included. Mean age was 13.9±2.9 years, ranging from 8-17 years. Etiology was traumatic in 2 cases, developmental in 2, and syndromic, Hirayama disease and post-laminectomy in 1 case each. Mean kyphosis correction was 36.80±19.30 (87%±21%) with a mean pre-operative kyphosis angle of 37.80±15.30 and mean immediate post-operative kyphosis angle of 3.70±8.70. Mean hospital stay duration was 10±6 days. Median follow-up duration was 36 months. Myelopathy improved in 5 cases at last follow-up. Six cases demonstrated bony fusion at a mean follow-up of 8.4±1.5 months., Conclusion: Significant immediate correction in pediatric cervical kyphosis may be achieved with a standalone posterior approach with proper planning and technique in selected cases. Inserting pedicle screws at strategic locations of implant construct offer better corrections and pull-out strength and maintain long-term stability resulting in higher arthrodesis rates. Larger studies with longer follow up are needed to further ascertain the role of standalone posterior cervical approaches in pediatric cervical kyphosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study examined the contributions of antenatal anxiety, depression, and partner violence to low birth weight (LBW) in infants and to sex-specific birth weight outcomes among mothers from a cohort in urban India., Methods: Data from 700 mothers from the PRAMMS cohort (Prospective Assessment of Maternal Mental Health Study) were used. Pregnant women were assessed in each trimester-T1, T2 and T3, for symptoms of anxiety, and depression as well as partner violence. Multivariate analyses were performed for the whole sample and then for male and female infants separately. The final multivariable logistic regression models were each built using a backward selection procedure and controlling for confounders. To accommodate longitudinally measured data, change in scores (T2-T1 and T3-T2) of anxiety and depression were included in the model., Results: Of the 583 women with a singleton live birth, birth weight was available for 514 infants  and LBW was recorded in 80 infants (15.6%). Of these, 23 infants were preterm. Overall, higher T1 Depression scores (OR: 1.11; 95% CI 1.040, 1.187) and an increase in both Depression scores (OR: 1.12; 95% CI 1.047, 1.195) from T1 to T2 and Anxiety scores (OR: 1.32; 95% CI 1.079, 1.603) between T2 and T3 were predictors of LBW. Female infants had a higher chance of LBW with increase in maternal anxiety between T1-T2 (OR: 1.69; 95% CI 1.053, 2.708) and T2-T3 (OR: 1.49; 95% CI 1.058, 2.086); partner violence during pregnancy just failed to reach conventional statistical significance (OR: 2.48; 95% CI 0.810, 7.581) in girls. Male infants had a higher chance of LBW with higher baseline depression scores at T1 (OR: 1.23; 95% CI 1.042, 1.452) and an increase in depression scores (OR: 1.25; 95% CI 1.060, 1.472) from T1 to T2., Conclusion: Increasing prenatal anxiety and depressive symptoms in different trimesters of pregnancy were associated with LBW with sex-specific patterns of association in this sample from a Low and Middle Income Country., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

The COVID-19 pandemic has forced hospitals to prioritize admissions. Epilepsy surgeries have been postponed at most centers. As the pandemic continues with no definite end in sight in the near future, the question arises until when such patients should be denied appropriate treatment. A 12-year-old child with left-sided Rasmussen's encephalitis with drug refractory epilepsy (DRE) presented at the height of the pandemic, with worsening of seizure frequency from 4-5/day to 20/day, with new-onset epilepsia partialis continua. She demonstrated features of progressive cognitive decline. The pros and cons of operating during the pandemic were discussed with the parents by a multidisciplinary team. She underwent endoscopic left hemispherotomy. Postoperatively she became seizure free but developed hospital-acquired mild COVID infection for which she was treated accordingly. Chosen cases of severe DRE, as the one illustrated above, who are deemed to benefit from surgery by a multidisciplinary team of physicians, should be re-categorized into the most severe class of patients and scheduled for surgery as soon as possible. The risk benefit ratio of the seizures being mitigated by surgery on one hand and possibility of acquiring COVID infection during hospital stay has to be balanced and a decision made accordingly., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The aim was to study surgical outcomes in hangman's fractures in paediatric and adolescent patients and to demonstrate evolution in posterior surgery from C1-C2-C3 fusion to C1 sparing techniques., Methods: Patients (aged ≤ 18 years) operated at a tertiary level centre between September 2011 to February 2018 with more than 1 year of follow-up were included. Neurological status, type of fracture, operating time, blood loss, follow-up, and complications were assessed., Results: Nine patients were included, with mean age mean of 16.45 years, with a mean follow-up of 42.78 months. Six patients having neurological deficit showed improvement. Two patients, one having undergone C1-C3 lateral mass screw rod fixation (LMSF) and other had C2 pedicle screw with C3 LMSF, developed kyphosis for which fixation was further extended caudally. One patient with an old hangman's fracture with reabsorbed axis pedicle underwent C2 body screw along with C3-C4 pedicle screw rod fixation and C2 pedicle reconstruction. All patients showed evidence of postoperative fusion., Conclusion: Hangman's fractures in young patients can be successfully managed via posterior fixation. In our centre, we have evolved in the direction of motion preservation at C1 C2 joint, along with 3 column stable fixation of the C2 pedicle. C2 pedicle reformation has allowed motion preserving surgery in complex fracture types. Extension of construct till C4 in selected cases is important to prevent postoperative kyphosis.

We report a 2-year-old child with congenital hydrocephalus who presented with a frontal meningocoele due to ventriculo-subgaleal fistula secondary to hydrocephalus (internal rupture), which gradually ruptured spontaneously through the skin. This case is unique given its association with occipital encephalocele and is only the second case to present with external rupture beyond the age of 1 year. Further, the ventricles have ruptured through the skull, after the closure of anterior fontanelle, into the subgaleal space. It challenges the present understanding that external rupture occurs only through an open anterior fontanelle.

Purpose: Pediatric vertebral hemangiomas (VH) are exceedingly rare benign and highly vascular tumours of the spine. There are no guidelines available for management of these patients in literature. Purpose of this study is to evaluate the role of intraoperative ethanol embolization, surgical decompression, and instrumented fusion in pediatric symptomatic VH with single-level involvement., Methods: Surgery consisted of intraoperative bilateral pedicular absolute alcohol injection and laminectomy at the level of pathology followed by a short-/long-segment instrumented fusion using pedicle screws and rod. Seven patients (mean age 14 ± 2.4 years, range 10-17 years, five females and two males) (age < 18 years) who were treated using this technique at our institute since March 2008 to December 2013 were enrolled in this retrospective study. Demographical, clinical, radiological, operative details, and postoperative events were retrieved from hospital records. During follow-up visits, clinical status and imaging were recorded. Outcome assessed with clinical and neurological outcome score of American Spinal Injury Association (ASIA) Impairment Scale., Results: Duration of symptoms ranged from 3 to 60 months (mean, 14.7 ± 20.4 months). Clinical features include myelopathy with motor and sensory involvement in all (five were paraplegic), back pain in two patients, and bladder involvement in two patients. The preoperative American Spinal Injury Association (ASIA) Impairment Scale (AIS) were B in five patients and C and D in one patient each. All had pan vertebral body VH with severe cord compression in the thoracic region on imaging study. Mean duration of surgery was 248.6 ± 60 minutes (range 195-310 min) and blood loss was 535 ml (range 200-1500 ml). Immediate embolization was achieved in all patients, which allowed laminectomy and soft tissue hemangioma removal relatively easy. Post surgery, at mean follow-up of 45.3 (±23.2) months (range 1-78 months), all patients showed improvement in power (sphincter improvement in two patients). ASIA were E in six patients and D in one patient at the last follow-up., Conclusion: The present study is the largest series of pediatric symptomatic VH. This procedure is a safe, efficient method to treat symptomatic pediatric VH with severe cord compression. It seems to serve the purpose of providing embolization, cord decompression, rigid fusion at the same sitting without adding new morbidity, and preventing excessive blood loss.

Purpose: Intracranial aneurysms in children are not as common as in adults and there are many differences in the etiology, demographic variables, aneurysm location, aneurysm morphological characteristics, clinical presentation, and outcome in pediatric and adult intracranial aneurysms., Methods: All children (≤18 years) suffering from intracranial aneurysm managed at our center from July 2001 through June 2013 were included in the study, and the details of these patients were retrieved from the computerized database of our hospital., Observations: A total of 62 pediatric patients were treated for 74 aneurysms during the study period and constituted 2.3% of all intracranial aneurysms treated during the same period. The mean age at presentation was 13.5 years. Headache (82%) was the commonest presenting feature; other symptoms included seizures (21%), ictal loss of consciousness (27%), and motor/cranial nerve deficits (22.6%). Computed tomogram revealed subarachnoid hemorrhage in 58% of patients. Eighty-two percent of aneurysms were in anterior circulation. Sixty-seven percent of aneurysms were complex aneurysms. Fifty-eight percent of patients underwent surgical intervention while 30% underwent endovascular procedures. Twenty-one percent of the patients developed vasospasm. There was no postoperative mortality. Favorable outcome was seen in 72% of the patients., Conclusions: Pediatric intracranial aneurysms are uncommon as compared to in adult patients. Seizures and cranial nerve involvement are seen more often as the presenting features in children. Posterior circulation aneurysms are more common in children, as are the internal carotid artery bifurcation aneurysms. There is high incidence of giant, posttraumatic, and mycotic aneurysms in children.

Introduction: Intracranial teratoma is a rare entity, accounting for 0.5 % of all intracranial tumors and 2-4 % of intracranial tumors in children., Objective: To study the demographic profile, clinical, radiological, and histopathological features of pediatric intracranial teratomas., Methods: All cases of intracranial teratoma diagnosed over an 11-year period (2001-2011) were retrieved from records of the departments of Neurosurgery and Neuropathology. Patients' demographic profile, clinical and radiological features and intraoperative findings were recorded., Results: We identified 43 cases of CNS teratoma, which included 8 adults and 35 children. Of the pediatric cases, 23 were spinal in location. Twelve cases of pediatric intracranial teratoma were identified (eight males, four females). Eight occurred in the first decade and four in the second decade (age range 3 days to 16 years). Third ventricle was the most common site (six cases). Four cases were seen in association with occipital encephalocele. All patients underwent surgery except for a newborn, which died 3 days after birth and was autopsied. Histologically, eight cases were classified as mature teratomas and four as immature teratomas. All four cases of immature teratoma were located in the posterior third ventricle., Conclusion: A diagnosis of teratoma should be considered in young children presenting with a solid-cystic tumor in the region of the posterior third ventricle. Teratomas located in the posterior third ventricle are more likely to have immature components. In view of the coexistence of teratoma with occipital encephalocele, thorough histopathological sampling of the excised sac and contents is advised.

Purpose: Papillary glioneuronal tumors (PGNT) have been recently included as a distinct entity in the WHO classification of tumors of the central nervous system. Their molecular pathogenesis is not clear. In the current study, we present the morphological, immunohistochemical, and molecular features of four cases of PGNT reported over the past 11 years., Methods: Over a period of 11 years (January 2000-February 2010), there were four cases of PGNT, which were reviewed for histomorphological features. TP53 and IDH1 mutations were assessed using antibodies against p53 protein and for mutant IDH1(R132H) protein, respectively. Immunohistochemistry was also performed for epidermal growth factor receptor (EGFR) protein. Fluorescence in situ hybridization assay was used for analyzing 1p/19q deletion status., Results: All the tumors showed the characteristic biphasic morphology. Rare findings included minigemistocyte-like cells in one, angiomatous areas in three, focal necrosis in one, and a high MIB-1 labeling index of 12 and 13 %, respectively, in two of the cases. All lacked EGFR, IDH1 expression, and 1p/19q deletions. Interestingly, antibody for p53 labeled the tumor cells, mainly those showing glial differentiation, in two cases. At a mean follow-up of 30 months, there was no evidence of disease progression except in one case which recurred after 24 months., Conclusion: PGNT are rare CNS neoplasms. Despite showing focal morphological features reminiscent of oligodendroglial tumors and presence of astrocytic component, they usually lack the common genetic alterations involved in the pathogenesis of gliomas. Multi-institutional pooling of cases may aid in elucidating their oncogenetic pathway.

While India has a long tradition of home-based spiritual and religious care of the dying, there has been no contemporary palliative care until relatively recently. The existing and planned palliative care services in India are presented, and future perspectives and the opportunities for training for both professionals and lay volunteers are discussed.