Pediatric neurosurgical medulloblastoma outcomes in La Paz, Bolivia: How a Lower Middle-Income Country (LMIC) institution in South America compares to the United States.

BACKGROUND: How pediatric medulloblastoma patients fare in Lower Middle-Income Country (LMICs) in South America is not well understood. Correspondingly, the aim of this study was to summarize the pediatric neurosurgical experience of an institution in La Paz, and compare outcomes to that of a generalized High Income Country (HIC) United States (US) experience. METHODS: A retrospective review of all pediatric neurosurgical medulloblastoma patients at the Children's Hospital of La Paz, Bolivia (Hospital del Niño "Dr. Ovidio Aliaga Uria") between 2014 and 2023 was conducted and compared to a generalized US experience abstracted from the US National Cancer Database (NCDB) and National Inpatient Sample (NIS) databases. Categorical, continuous and survival data were statistically summarized and compared. RESULTS: A total of 24 pediatric medulloblastoma patients underwent neurosurgical treatment at the Hospital del Niño. In this La Paz cohort, there were 15 (63%) males and 9 (38%) females, with a mean age of 5.6 years old at diagnosis. The majority of patients underwent subtotal resection (STR, 79%), while the remaining patients underwent biopsy only. Ten (42%) patients expired during their hospitalization, and mean length of stay overall was 39 days. Only 8 (33%) patients received adjuvant treatment after surgery. Median overall survival from diagnosis in the La Paz cohort was 1.9 months. Compared to the US databases, the La Paz cohort experienced significantly more emergency room admissions for surgery, less gross total resection, more STR, more return to operating room for ventriculoperitoneal shunting, more bacteremia, more tracheostomy procedures, more percutaneous gastrostomy placements, longer lengths of stay, less adjuvant chemotherapy, less radiation therapy, shorter follow-up, and ultimately, significantly shorter overall survival (all P < 0.050). CONCLUSIONS: Pediatric neurosurgical medulloblastoma outcomes at the Children's Hospital of La Paz, Bolivia are significantly inferior to that of a generalized US experience. Future research is required to identify institution- and country-specific initiatives to improve discrepancies between institutions in LMICs in South America compared to HICs.

The epidemiologic associations of food availability with national incidence and mortality rates of pediatric central nervous system tumors.

BACKGROUND: The epidemiology of central nervous system (CNS) tumors in pediatric patients worldwide continues to be defined. To date, there has been no evaluation of how national food availability may associate with the incidence and mortality of these tumors. Correspondingly, the aim of this study was to define if such associations exist. METHODS: The most updated incidence and mortality rates of CNS tumors in pediatric patients were abstracted by country from the Global Burden of Disease database. Data regarding food availability parameters were identified and abstracted from the Food Systems Dashboard database. Associations were tested using univariate and multivariate regression analyses. RESULTS: There were sufficient data in a total of 175 countries worldwide describing the required outcomes. Median incidence and mortality rates across these countries were 1.63 per 100,000 and 0.80 per 100,000, respectively. Higher incidence rates of pediatric CNS tumors were statistically associated with lower availability of fruit and vegetables (P = 0.02), higher average protein supply (P < 0.01), lower share of dietary energy from cereal and roots (P < 0.01), lower supply of meat (P < 0.01), lower supply of nuts and seeds (P < 0.01), lower supply of vegetable oils (P < 0.01), and higher supply of vegetables (P < 0.01). Higher mortality rates due to pediatric CNS tumors were statistically associated with lower availability of fruit and vegetables (P = 0.048), lower supply of fish (P = 0.046), and lower supply of nuts and seeds (P = 0.04). When categorizing countries based on income status, there was a decrease in significant associations found more pronounced in low-middle income countries. CONCLUSIONS: There are many novel associations between national food availability and the incidence and mortality rates of pediatric CNS tumors across the world, which may be more pronounced and divergent in low-middle income countries. A greater understanding is needed to identify what specific components of the significant parameters influence these trends and how public health efforts may best address these associations to improve overall outcomes.

Racial disparities in pediatric malignant glioma management: current state of affairs in the United States.

BACKGROUND: The diagnosis of malignant glioma confers a poor prognosis in the pediatric population. In the adult demographic, racial disparities exist with respect to access to care and survival. Yet to date no efforts have been made to characterize racial disparities in the care of malignant pediatric gliomas. Correspondingly, the aim of this study was to understand if racial disparities exist in the setting of malignant pediatric gliomas. METHODS: All pediatric malignant gliomas patients with known race status (White, Black, Other) in the US National Cancer Database (NCDB) between the years 2005-2016 were retrospectively reviewed. Demographic, socioeconomic and clinical data were then abstracted and analyzed by comparison and regression techniques. RESULTS: A total of 1803 pediatric malignant glioma cases were identified, with 48% female and a median age of 8 years old. Brainstem locations were reported in 48% of cases. Socioeconomically, there were statistically significant differences with respect to insurance status, yearly income, household education level and metropolitan residences between the racial groups (all P < 0.01). With respect to treatment, there was statistical difference in the proportion of patients treated with surgical resection (White 43% vs Black 34% vs Other 37%, P = 0.02). There were no differences between race groups for radiation therapy (P = 0.73) or chemotherapy (P = 0.12). The odds of surgical resection were significantly less in the Black group compared to the White group (OR 0.69, P < 0.01), although there was no difference in overall survival between the two groups in those treated with (P = 0.44) or without (P = 0.27) surgical resection. Primary associations of surgical resection in the Black group were brainstem location (P < 0.05) and lower yearly household income quartiles (P < 0.05). CONCLUSIONS: Racial disparities exist amongst the management of pediatric malignant gliomas, with undefined impact on survival and quality of life. In this perspective, we identified associations between Black patients and access to surgical treatment. Understanding that there are many elements to patient care, including quality of life, should encourage all clinicians and carers to consider racial disparities appropriately when managing malignant pediatric glioma patients.

Disseminated diffuse midline glioma associated with poorly differentiated orbital lesion and metastases in an 8-year-old girl: case report and literature review.

BACKGROUND: Disseminated diffuse midline glioma (DMG) is a devastating diagnosis. Molecular subtyping has increased our understanding of this tumor. CASE: Here, we report the case of an 8-year-old girl who presented with symptoms of brainstem dysfunction and was found to have disseminated DMG with lesions in the pons, thalamus and bilateral temporal lobes. Molecular subtyping of the temporal lobe tumor tissue was consistent with H3 K27me3 loss and EZHIP overexpression, falling under the newly designated "H3 K27-altered" AQ5WHO subtype of DMG. Pathology from biopsy of the orbital lesion showed poorly differentiated rhabdoid-like disseminated tumor cells. The patient went on to develop lesions in the peritoneum, infratemporal fossa, and along the lumbosacral nerve roots. CONCLUSION: This unique case illustrates the aggressive behavior of H3 K27-altered tumors and their potential to metastasize.

Clinical complications of surviving gunshot wounds to the head in children and adolescents: the Miami experience.

BACKGROUND: Gunshot wounds (GSWs) to the head in the pediatric population are both rare and devastating, with the clinical course of pediatric survivors poorly understood. Correspondingly, the aim of this study was to summarize the clinical complications clinicians can expect of survivors of GSW to the head in children and adolescents in hospital and after discharge. METHODS: A retrospective review of our Level 1 trauma center database between 2011 and 2021 was performed. Clinical data was extracted for those patients aged ≤ 18 years old who survived initial hospitalization with at least one documented follow-up. Categorical data were then compared using Chi-squared test. RESULTS: A total of 19 pediatric survivors of GSW to the head satisfied all selection criteria with an average age was 15.3 years. The majority of cases were isolated head injuries (63%), with an average Glasgow Coma Score (GCS) of 11.9. Bullet trajectory was intraparenchymal in 11 (58%) cases and extraparenchymal in 8 (42%) cases, with 15 (79%) patients treated by surgical intervention. A total of 13 (68%) patients experienced a complication during their hospitalization, with the most common being sympathetic hypertension and endocrinologic salt wasting, each occurring in 5 (26%) patients. With respect to complication categories, the intraparenchymal patients experienced statistically more complications than extraparenchymal patients that were infectious (54% vs 0%, P = 0.01) and sympathetic (45% vs 0%, P = 0.03) in nature. However, with respect to overall neurologic (P = 0.24), endocrinologic (P = 0.24), and traumatic (P = 0.24) complications, their incidences were statistically comparable. All patients were successfully discharged on average post-injury day 22 with an average GCS of 14.0. Mean follow-up for the cohort was 42.6 months, with an average GCS of 14.3. A total of 6 (32%) patients experienced a complication relatable to their initial GSW injury after discharge. The most common individual complication was new-onset seizures in 3 (16%) patients. CONCLUSIONS: Survivors of pediatric GSW to the head can experience multi-systemic complications during both initial hospitalization and afterwards, and bullet trajectory involving the parenchyma may be associated with specific complications more than others. Dedicated inpatient management and outpatient follow-up involving surveillance for complications across all systems, not just neurological, are recommended to ensure patients receive the best care possible.

Cervical intramedullary teratoma: a case report and systematic review of the literature.

Here, we report a case of a 3-year-old female who presented to clinic with an enlarging mass in the posterior cervical midline. The mass was present since birth and demonstrated no cutaneous stigmata. Plain film, CT, and MRI of the cervical spine (C3-C5) revealed enlargement of the spinal canal, soft tissue calcification, spinal dysraphism, and an intramedullary, predominantly fatty, mass. The mass had associated calcifications and a highly proteinaceous cyst. Surgical resection of the spinal lesion was subsequently performed. Histopathological evaluation revealed a mature teratoma. Cervical spinal teratomas in the pediatric population are rare entities with few cases currently reported in the literature. We conducted a systematic review to outline the current evidence detailing cases of intramedullary spinal cord teratomas. Six articles were included for final review. All patients in the included articles underwent maximal surgical resection with one patient also receiving chemotherapy and radiation. With our report, we aim to add to the literature on cervical intramedullary spinal cord teratomas in the pediatric population.

Distinct survival and clinical profile of infantile glioblastoma: insights from a national database.

BACKGROUND: The diagnosis of glioblastoma (GBM) in infants aged ≤ 1 year is extremely rare, and its comparability to the more common adult diagnosis is underexplored. Correspondingly, the objective of this study was to interrogate a national cancer database to elucidate the typical survival and clinical profile of this demographic. METHODS: All GBM patients aged ≤ 1 year in the U.S. National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival (OS) was modeled using Kaplan-Meier and Cox regression analyses. RESULTS: A total of 86 patients satisfied criteria for entry into study, making up 0.08% of all GBM diagnoses in the database. There were 32 (37%) females and 54 (63%) males. Irrespective of treatment, median OS was 67.3 months (95% CI, 46-91), which was distinct from all other ages and pediatric age groups. There were 74 (86%) treated by surgery, 51 (59%) treated by chemotherapy, and 17 (20%) treated by radiation therapy. Multivariable analysis demonstrated that Hispanic status (HR = 3.41, P = 0.02) and the presence of comorbidity (HR = 3.24, P = 0.01) independently predicted shorter OS, whereas treatment with chemotherapy (HR = 0.18, P < 0.01) independently predicted longer OS. Neither extent of surgery nor radiation therapy demonstrated independent statistical significance. CONCLUSION: Infantile GBM should be viewed as a distinct GBM entity with a longer OS than other pediatric and adult patients. Chemotherapy is a statistically significant component in the treatment of this demographic, and the value of surgical treatment is likely universal. Future studies into understanding the biological and genetic profile of infantile GBM are needed to advance both pediatric and adult fields.

Myelomeningocele Including Fetal Prescription.

Myelomeningocele (MMC) is one of the most common birth defects, affecting 0.2 to 0.4 per 1,000 live births in the United States. The most strongly associated risk factor is low folate level in pregnancy. For this reason, 0.4- to 1.0-mg supplementation with folic acid is recommended in all pregnancies, and high-risk pregnancies are recommended to supplement with 4.0 mg of folic acid daily. The mechanism behind the development of MMC is believed to be failure of the caudal end of the neural tube to close during primary neurulation. Screening for MMC is achieved by using α-fetoprotein levels in maternal serum or amniocentesis in the first and second trimesters of pregnancy. Ultrasonography and fetal magnetic resonance imaging are used to confirm the presence of MMC as well as the location and size of the defect. Based on the results of the Management of Myelomeningocele Study, fetal repair is performed between 23 weeks and 25 weeks and 6 days of gestational age for appropriate candidates. Postnatal repair is more common and is performed 24 to 72 hours after birth. In general, patients with lesions at lower anatomical levels have a better prognosis. Most children with MMC will have neurogenic bladder and bowel dysfunction that affect the patient's and the caregiver's quality of life. Patients with higher levels of mobility, better familial support, and higher economic status report improved quality of life compared with other patients with MMC.

Age of diagnosis clinically differentiates atypical teratoid/rhabdoid tumors diagnosed below age of 3 years: a database study.

BACKGROUND: Atypical teratoid/rhabdoid tumor (ATRT) is a rare and largely pediatric diagnosis, with poor survival. Diagnosis below the age of 3 years is characteristically seen as a poor prognostic sign. However, elucidating if clinical differences exist within this niche age group has never been attempted before. Correspondingly, we sought to characterize clinical profile of ATRT diagnoses before the age of 3 years based on separate ages of diagnosis. METHODS: All pediatric ATRT patients aged < 3 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Age groups were divided based on diagnoses at ages 0-1 years in group 1, 1-2 years in group 2, and 2-3 years in group 3. Data were summarized, and overall survival (OS) was modeled using Kaplan-Meier and Cox regression analyses. RESULTS: A total of 354 ATRT diagnoses were made before the age of 3 years, with surgery used in 316 (89%) cases, chemotherapy in 242 (68%) cases, and radiation therapy in 118 (33%) cases. In terms of diagnosis age, there were 153 (43%) in group 1, 137 (39%) in group 2, and 64 (18%) in group 3. With respect to OS, median value was 9.9 months in group 1, 28.4 months in group 2, and 15.9 months in group 3. Upon multivariate analysis, receiving radiation therapy was the only parameter shared amongst all three groups as independently prognostic of longer OS (HR 0.53, P = 0.01 in group 1; HR 0.34, P < 0.01 in group 2; HR 0.31, P < 0.01 in group 3). In group 1, surgery (HR 0.47, P < 0.01) and chemotherapy (HR 0.44, P < 0.01) were also independently prognostic of longer OS. In group 3, multiple socioeconomic parameters were identified to independently predict longer OS. There were no additional predictive parameters identified in group 2. CONCLUSION: Although ATRT diagnosed before the age of 3 is typically viewed a poor prognostic age category, our findings demonstrate that the clinical profile of this pediatric niche is highly heterogeneous based on age of diagnosis. Survival of only those diagnosed between 0 and 1 years is independently prognosticated by all three treatment modalities; patients diagnosed between 1 and 2 years trend towards longest survival, and socioeconomic parameters are most influential in those diagnosed between 2 and 3 years.

Indeterminate Cell Histiocytosis of the Spine: A Case Report.

INTRODUCTION: Indeterminate cell histiocytosis is a rare neoplastic disease characterized by proliferation of dendritic cells that share morphologic and immunophenotypic features of Langerhans cells and non-Langerhans histiocytes. ICH lesions are typically restricted to the skin; however, there have been rare reports of extracutaneous and systemic involvement. CASE PRESENTATION: We describe a case of a 13-year-old female presenting with complaints of worsening lower back pain for 1 year. CT and MRI of the lumbar spine demonstrated a well-defined bony, lytic, expansile lesion of the posterior and mid-endplate of the L4 vertebra with mass effect on the thecal sac. The patient underwent L3-L5 decompression and fusion with surgical excision of the vertebral body tumor. Microscopic examination of the lesion showed benign fibrohistiocytic proliferation with giant cells, and immunohistochemical staining revealed a phenotype consistent with indeterminate cell histiocytosis (S100+ CD1a+ langerin-). DISCUSSION/CONCLUSION: ICH is an extraordinarily rare neoplastic disease of dendritic cells that has a poorly understood pathogenesis. This case expands the spectrum of potential ICH extracutaneous involvement to now include the spine, a location previously undocumented in the literature in the pediatric population.

Pediatric Spinal Cord Diseases.

Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.

Endoscopic third ventriculostomy inpatient failure rates compared with shunting in post-hemorrhagic hydrocephalus of prematurity.

PURPOSE: Endoscopic third ventriculostomy (ETV) has gained traction as a method for treating post-hemorrhagic hydrocephalus of prematurity (PHHP) in an effort to obviate lifelong shunt dependence in neonates. However, data remains limited regarding inpatient failures. METHODS: A retrospective analysis of the NIS between 1998 and 2014 was performed. Discharges with age < 1 year and ICD-9-CM codes indicating intraventricular hemorrhage of prematurity (772.1x) and ETV/shunt (02.22 and 02.3x) were included. Patients with ICD-9-CM codes for ventricular drain/reservoir (02.21) were excluded to prevent confounding. Time trend series plots were created. Yearly trends were quantified using logarithmic regression analysis. Kaplan-Meier curves were utilized to analyze time to treatment failure. Time to failure for each treatment was compared using log-rank. RESULTS: A total of 11,017 discharges were identified. ETV was more likely to be utilized at < 29 weeks gestational age (p = 0.0039) and birth weight < 1000 g (p = 0.0039). Shunts were less likely to fail in older and heavier newborns (OR 0.836 p = 0.00456, OR 0.828 p = 0.0001, respectively). Those initially shunted had lower failure rates compared with ETV (OR 0.44, p < 0.0001) but time to failure was longer with ETV (p = 0.04562). 79.5% of ETVs that failed were shunted after the first failure. Shunts were much less likely to undergo ETV if they failed (OR 0.21, p < 0.0001). Higher grade IVH was predictive of shunt failure but not ETV (OR 2.36, p = 0.0129). CONCLUSIONS: Although ETV can be effective in PHHP, it has a much higher initial failure rate than shunting and should thus be chosen based on a multifactorial approach.

Carotid Body Tumor Microenvironment.

Carotid body tumors (CBTs) are rare paragangliomas, comprising 0.5% of all head and neck tumors, and 65% of head and neck paragangliomas. A majority of CBTs occur sporadically, while 15% are familial or hyperplastic in the setting of chronic hypoxia. They usually present as unilateral, well-circumscribed rubbery masses, arising at the level of the carotid bifurcation. A majority of CBTs are painless and therefore may evade diagnosis for months to years. Symptomatic lesions occur due to progressive cranial nerve IX, X, or XII dysfunction, manifesting as hoarseness, dysphagia, vertigo, coughing, or odynophagia. Other local symptoms include neck discomfort, pulsatile tinnitus, hearing loss, or carotid sinus syndrome. Appropriate workup includes a thorough physical exam followed by radiographic imaging, vascular studies, and biochemical workup with 24 h urinary catecholamine or metanephrine analysis. The management of these tumors, which involves standalone surgical resection or following embolization for larger and more vascular tumors, will also be discussed.

Pediatric and adult H3 K27M-mutant diffuse midline glioma treated with the selective DRD2 antagonist ONC201.

BACKGROUND: H3 K27M-mutant diffuse midline glioma is a fatal malignancy with no proven medical therapies. The entity predominantly occurs in children and young adults. ONC201 is a small molecule selective antagonist of dopamine receptor D2/3 (DRD2/3) with an exceptional safety profile. Following up on a durable response in the first H3 K27M-mutant diffuse midline glioma patient who received ONC201 (NCT02525692), an expanded access program was initiated. METHODS: Patients with H3 K27M-mutant gliomas who received at least prior radiation were eligible. Patients with leptomeningeal spread were excluded. All patients received open-label ONC201 orally once every week. Safety, radiographic assessments, and overall survival were regularly assessed at least every 8 weeks by investigators. As of August 2018, a total of 18 patients with H3 K27M-mutant diffuse midline glioma or DIPG were enrolled to single patient expanded access ONC201 protocols. Among the 18 patients: seven adult (> 20 years old) and seven pediatric (< 20 years old) patients initiated ONC201 with recurrent disease and four pediatric patients initiated ONC201 following radiation, but prior to disease recurrence. FINDINGS: Among the 14 patients with recurrent disease prior to initiation of ONC201, median progression-free survival is 14 weeks and median overall survival is 17 weeks. Three adults among the 14 recurrent patients remain on treatment progression-free with a median follow up of 49.6 (range 41-76.1) weeks. Among the 4 pediatric patients who initiated adjuvant ONC201 following radiation, two DIPG patients remain progression-free for at least 53 and 81 weeks. Radiographic regressions, including a complete response, were reported by investigators in a subset of patients with thalamic and pontine gliomas, along with improvements in disease-associated neurological symptoms. INTERPRETATION: The clinical outcomes and radiographic responses in these patients provide the preliminary, and initial clinical proof-of-concept for targeting H3 K27M-mutant diffuse midline glioma with ONC201, regardless of age or location, providing rationale for robust clinical testing of the agent.

Extracranial extra-CNS spread of embryonal tumor with multilayered rosettes (ETMR): case series and systematic review.

BACKGROUND: Embryonal tumors with multilayered rosettes (ETMR) is a rare variant of embryonal tumor of infancy with nearly 200 cases reported in the literature. Leptomeningeal spread of this tumor is well known; however, extracranial metastasis has been sparsely reported in the literature. METHODS: Our study was divided into two sections: (1) We conducted a retrospective review of our patient series of ETMR and screened for patients with evidence of ETMR over the last 10 years 2007-2017 at a single tertiary referral pediatric hospital, and (2) we conducted a systematic review according to PRISMA guidelines of all reported cases of ETMR to determine the incidence of extracranial metastasis and treatment paradigms. RESULTS: Here we report three cases of extracranial non-CNS spread of ETMR and conduct a systematic review of ETMR to improve our understanding of ETMR metastases and treatment paradigms. In our systematic review (n = 204), median overall survival was less than 1 year with 44.1% children surviving over 1 year. Previously, only five cases of extracranial metastasis of ETMR have been reported. CONCLUSIONS: Our case series (n = 3) and review demonstrate that these tumors may behave like soft tissue sarcomas and may be susceptible to tumor seeding through surgical manipulation or by CSF (ventriculoperitoneal shunt). Surgery for tumor recurrence may offer an improved local disease control, but preventative measures such as meticulous surgical resection may be necessary to reduce intraoperative contamination.

Awake Craniotomy in Pediatric Patients: A Meta-analysis of Operative Outcomes.

BACKGROUND: Awake craniotomy allows neurosurgeons to make critical decisions when operating in eloquent regions of the brain. The phenomenon of "waking up" during surgery is underexplored in pediatric patients, and the operative outcomes following awake craniotomy are not well understood. Correspondingly, the aim of this study was to quantitatively aggregate the contemporary metadata regarding the operative outcomes of awake craniotomy when used in the pediatric setting. METHODS: Multiple electronic databases from inception to June 2023 were searched following PRISMA guidelines. Respective cohort-level outcomes were then abstracted and pooled by means of meta-analysis utilizing random-effects modeling, and trends evaluated by meta-regression analysis. RESULTS: There were 4 observational studies that satisfied all selection criteria, describing a total cohort of 57 pediatric patients undergoing awake craniotomy. There were 34 (60%) male patients with a median age of 14 years old, with lesions on the left side in 80% of cases when reported. Meta-analysis demonstrated pooled incidences of intraoperative complication to be 17% (95% CI 4%-37%), the need to convert cases to general anesthesia to be 2% (95% CI 0%-9%), immediate postoperative complication to be 18% (95% CI 6%-33%), and long-term complications to be 6% (95% CI 0%-15%). The most common intraoperative complication reported was seizure. Certainty of these estimates were very low due to limited metadata. Meta-regression did not indicate any trend bias due to study and cohort parameters. CONCLUSIONS: Multiple studies have demonstrated the feasibility of the awake craniotomy approach in pediatric patients. The risks for intraoperative and postoperative complications are non-zero, with their incidences trending towards incidences seen in the adult demographic. It is likely formal neuropsychologic preparation and follow-up will increase the candidacy and success of this approach in the future.

Assessing pediatric neurosurgery capacity in La Paz, Bolivia: an illustrative institutional experience of a lower-middle-income country in South America.

OBJECTIVE: The current pediatric neurosurgery capacity in lower-middle-income countries (LMICs) in South America is poorly understood. Correspondingly, the authors sought to interrogate the neurosurgical inpatient experience of the sole publicly funded pediatric hospital in one of the largest regional departments of Bolivia to better understand this capacity. METHODS: A retrospective review of all neurosurgical procedures performed at the Children's Hospital of La Paz, Bolivia (Hospital del Niño "Dr. Ovidio Aliaga Uria") between 2019 and 2023 was conducted after institutional approval using a recently implemented national electronic medical record system. RESULTS: A total of 475 neurosurgical admissions satisfied inclusion for analysis over the 5-year span. The majority of admissions were from within the La Paz Department (87%) via the emergency department (77%), without private insurance (83%). The most common indications for neurosurgical intervention were trauma (35%), followed by hydrocephalus (28%), congenital disease (12%), infection (5%), and craniosynostosis (3%). Overall, the median age at time of surgery was 2.0 years, and the median operating time was 1.5 hours with a minority of intraoperative complications (2%). The most common inpatient complication was unplanned return to the operating room (19%), most commonly seen in congenital indications. At final discharge, the median postoperative length of stay was 10 days. Twenty-seven (6%) of the 475 patients died during hospitalization, most commonly seen in tumor indications. Of the 448 patients who were discharged, 299 (67%) returned for at least one follow-up appointment. CONCLUSIONS: There is restricted breadth in neurosurgical indications and outcomes achievable at the Children's Hospital of La Paz, Bolivia. As such, the capacity of pediatric neurosurgery at institutions in LMICs in South America such as this one is very limited. Identifying and prioritizing actionable interventions to improve this capacity is institution- and LMIC-dependent, and as such, future efforts will need to be tailored appropriately.

Unraveling the complexity of the senescence-associated secretory phenotype in adamantinomatous craniopharyngioma using multimodal machine learning analysis.

BACKGROUND: Cellular senescence can have positive and negative effects on the body, including aiding in damage repair and facilitating tumor growth. Adamantinomatous craniopharyngioma (ACP), the most common pediatric sellar/suprasellar brain tumor, poses significant treatment challenges. Recent studies suggest that senescent cells in ACP tumors may contribute to tumor growth and invasion by releasing a senesecence-associated secretory phenotype. However, a detailed analysis of these characteristics has yet to be completed. METHODS: We analyzed primary tissue samples from ACP patients using single-cell, single-nuclei, and spatial RNA sequencing. We performed various analyses, including gene expression clustering, inferred senescence cells from gene expression, and conducted cytokine signaling inference. We utilized LASSO to select essential gene expression pathways associated with senescence. Finally, we validated our findings through immunostaining. RESULTS: We observed significant diversity in gene expression and tissue structure. Key factors such as NFKB, RELA, and SP1 are essential in regulating gene expression, while senescence markers are present throughout the tissue. SPP1 is the most significant cytokine signaling network among ACP cells, while the Wnt signaling pathway predominantly occurs between epithelial and glial cells. Our research has identified links between senescence-associated features and pathways, such as PI3K/Akt/mTOR, MYC, FZD, and Hedgehog, with increased P53 expression associated with senescence in these cells. CONCLUSIONS: A complex interplay between cellular senescence, cytokine signaling, and gene expression pathways underlies ACP development. Further research is crucial to understand how these elements interact to create novel therapeutic approaches for patients with ACP.

Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome.

INTRODUCTION: Posterior fossa tumors are the most common brain tumor of children. Aggressive resection correlates with long-term survival. A high incidence of posterior fossa syndrome (PFS), impairing the quality of life in many survivors, has been attributed to damage to bilateral dentate nucleus or to cerebellar output pathways. Using diffusion tensor imaging (DTI), we examined the involvement of the dentothalamic tracts, specifically the superior cerebellar peduncle (SCP), in patients with posterior fossa tumors and the association with PFS. METHODS: DTI studies were performed postoperatively in patients with midline (n = 12), lateral cerebellar tumors (n = 4), and controls. The location and visibility of the SCP were determined. The postoperative course was recorded, especially with regard to PFS, cranial nerve deficits, and oculomotor function. RESULTS: The SCP travels immediately adjacent to the lateral wall of the fourth ventricle and just medial to the middle cerebellar peduncle. Patients with midline tumors that still had observable SCP did not develop posterior fossa syndrome (N = 7). SCPs were absent, on either preoperative (N = 1, no postoperative study available) or postoperative studies (N = 4), in the five patients who developed PFS. Oculomotor deficits of tracking were observed in patients independent of PFS or SCP involvement. CONCLUSION: PFS can occur with bilateral injury to the outflow from dentate nuclei. In children with PFS, this may occur due to bilateral injury to the superior cerebellar peduncle. These tracts sit immediately adjacent to the wall of the ventricle and are highly vulnerable when an aggressive resection for these tumors is performed.