Pediatric hemispheric cerebellar low-grade gliomas: clinical approach, diagnosis, and management challenges-experience at a tertiary care children's hospital.

PURPOSE: This study aims to provide an exhaustive analysis of pediatric low-grade gliomas (pLGGs) in the cerebellar hemispheres, focusing on incidence, clinical characteristics, surgical outcomes, and prognosis. It seeks to enhance understanding and management of pLGGs in the pediatric population. METHODS: We conducted an observational, descriptive, retrospective, and cross-sectional study at a pediatric hospital, reviewing medical records of 30 patients with cerebellar hemispheric pLGGs treated from December 2014 to January 2023. Data collection included demographics, clinical presentation, imaging findings, surgical approach, postoperative complications, histopathological diagnosis, hydrocephalus management, and follow-up. Molecular markers and adjuvant therapies were also analyzed. RESULTS: The cohort predominantly presented with cerebellar symptoms, with 60% showing hydrocephalus at diagnosis. MRI with gadolinium was crucial for diagnosis. Surgical focus was on achieving gross total resection (GTR), accomplished in 70% of cases. Postsurgical hydrocephalus was less common, and cerebellar mutism was not reported. While a complete molecular analysis was not performed in all cases, available data suggest significant influence of molecular markers on prognosis and therapeutic options of pLGGs. CONCLUSIONS: This study highlights the unique clinical and molecular characteristics of cerebellar hemispheric pLGGs in children. The lower incidence of postoperative hydrocephalus and absence of cerebellar mutism are notable findings. Emphasizing a multidisciplinary approach, our findings contribute to a deeper understanding of pediatric pLGGs, underscoring the need for personalized treatment strategies and vigilant follow-up.

Global Neurosurgery at the 76th World Health Assembly (2023): First Neurosurgery-Driven Resolution Calls for Micronutrient Fortification to Prevent Spina Bifida.

Since 2018, a neurosurgery delegation has been actively engaged and consistently present at the World Health Assembly. Recognizing the growing impact of neurosurgical diseases, the neurosurgery delegation participated in the 76th World Health Assembly in May 2023, advocating for timely, safe, and affordable global neurosurgical care. The delegation focused on forging new collaborations, strengthening the World Health Organization-World Federation of Neurosurgical Societies official relations, and actively supporting resolutions that impact the neurosurgical patients. However, there is a long advocacy journey ahead to address unmet neurosurgical needs. Patient-centered advocacy is an inherent task of our profession and the essence of the Global Neurosurgery Bogota Declaration of 2016. The highlight of the 76th World Health Assembly was the adoption of the first neurosurgery-driven resolution calling for micronutrient fortification to prevent spina bifida and other micronutrient deficiencies. For the last 4 years, the Global Alliance for Prevention of Spina Bifida, a group spearheaded by neurosurgeons, advocated for spina bifida prevention. This Alliance collaborated with many stakeholders, notably, the Colombian government to promote the resolution: "Accelerating efforts for preventing micronutrient deficiencies and their consequences, including spina bifida and other neural tube defects, through safe and effective food fortification." This is a proud milestone for the neurosurgical profession. There are many strategies available for neurosurgeons, when working together with elected leaders, other stakeholders, and allied professionals, to implement initiatives that can prevent future cases of spina bifida and other neurological disorders and reduce the burden of neurosurgical disease.

Toxoplasma gondii infections in pediatric neurosurgery.

Toxoplasma gondii is a parasite that is estimated to infect one-third of the world's population. It is acquired by ingesting contaminated water and food specially undercooked meat, contact with domestic or wild feline feces, and during pregnancy by transplacental transmission.Immunocompetent hosts are usually asymptomatic, and infection will be self-limited, while those patients whose immune system is debilitated by HIV infection, immunosuppressive therapy, long-term steroid treatment, and fetuses infected during gestation will show evidence of systemic activity which is more severe in the central nervous system and eyes due to insufficient immune response caused by their respective blood barriers. Congenital toxoplasmosis has an estimated incidence of 8% in mothers who were seronegative at the beginning of their pregnancy. Infection in the first trimester may result in spontaneous abortion or stillbirth; however, it is estimated that the highest risk for vertical transmission is during the second and third trimesters when blood flow and placenta thickness favor parasitic transmission.Congenital toxoplasmosis can be detected with periodic surveillance in endemic areas, and with appropriate treatment, the risk of vertical transmission can be reduced, and the severity of the disease can be reversed in infected fetuses.While most infected newborns will show no evidence of the disease, those who suffer active intrauterine complications will present with cerebral calcifications in 8-12% of cases, hydrocephalus in 4-30%, and chorioretinitis in 12-15%. Also, seizure disorders, spasticity, and varying degrees of neurocognitive deficits can be found in 12%.Four distinct patterns of hydrocephalus have been described: aqueductal stenosis with lateral and third ventricle dilatation, periforaminal calcifications leading to foramen of Monro stenosis with associated asymmetrical ventricle dilatation, a mix of aqueductal and foramen of Monro stenosis, and overt hydrocephalus without clear evidence of obstruction with predominant dilatation of occipital horns (colpocephaly).While all patients diagnosed with congenital toxoplasmosis should undergo pharmacological treatment, those presenting with hydrocephalus have traditionally been managed with CSF shunting; however, there are reports of at least 50% success when selected cases are treated with endoscopic third ventriculostomy. Successful hydrocephalus management with appropriate treatment leads to better intellectual outcomes.

Basic Science Perspective on Engineering and Modeling the Large Airways.

The airway epithelium provides a physical and biochemical barrier playing a key role in protecting the lung from infiltration of pathogens and irritants and is, therefore, crucial in maintaining tissue homeostasis and regulating innate immunity. Due to continual inspiration and expiration of air during breathing, the epithelium is exposed to a plethora of environmental insults. When severe or persistent, these insults lead to inflammation and infection. The effectiveness of the epithelium as a barrier is reliant upon its capacity for mucociliary clearance, immune surveillance, and regeneration upon injury. These functions are accomplished by the cells that comprise the airway epithelium and the niche in which they reside. Engineering of new physiological and pathological models of the proximal airways requires the generation of complex structures comprising the surface airway epithelium, submucosal gland epithelium, extracellular matrix, and niche cells, including smooth muscle cells, fibroblasts, and immune cells. This chapter focuses on the structure-function relationships in the airways and the challenges of developing complex engineered models of the human airway.

Pre and postnatal care characteristics and management features of children born with myelomeningocele in the post-folate fortification era of staple foods in Costa Rica (2004-2022).

PURPOSE: To describe the clinical characteristics of pre- and postnatal care of children born with myelomeningocele in Costa Rica from 2004 to 2022 after the introduction of mandatory fortification of four major staple foods, describing the clinical features of this cohort including the size of the meningomyelocele, neurological level, presence of symptomatic Chiari II at birth, kyphosis, and the severity of hydrocephalus requiring cerebrospinal fluid (CSF) shunting. These results were compared against the pre-fortification historical data to determine favorable outcomes from this health policy. METHODS: We performed a retrospective review of the clinical records of patients with myelomeningocele at the spina bifida clinic from the National Children's Hospital of Costa Rica who were born between 2004 and 2022, a period when staple food fortification was implemented in the country for four food staples (wheat and corn flour, rice, and dairy products). Pre and postnatal care data pertaining to the number of obstetric ultrasound studies, the trimester in which they were performed, the detection of myelomeningocele and associated hydrocephalus, gestational age and route of delivery, neurological level, myelomeningocele defect size, associated kyphosis and symptomatic Chiari II malformation at birth, time periods of myelomeningocele repair, and CSF shunting were examined. A descriptive comparative frequency analysis between myelomeningocele (MMC) defect size, CSF shunt insertion, symptomatic Chiari II, and kyphosis between the different functional levels was performed with the estimation of the Fisher's exact chi-square test by contingency tables, and 0.05 was set as significance level. Additionally, the postnatal features of this cohort were compared against pre-fortification historical data obtained from 100 live-born patients between 1995 and 1996. RESULTS: A total of 215 patients that were live born between 2004 and 2022 were eligible for analysis with a follow up ranging from 1 to 19 years (median follow up: 7.9 years). Among 99.1% of the mothers of patients who had prenatal consultations, 95.8% had an average of 3.8 obstetric ultrasound studies which led to a 59% prenatal detection rate of myelomeningocele. The pre and post fortification features showed a male/female ratio that changed from 0.92 to 1.25 respectively. Among these newborns, there was an increase from 54 to 64% cesarean sections as method for delivery. Only 26% of the pre fortification patients had the MMC defect repaired in the first 24 h, 32% from 24 to 72 h, 20% from 72 h to 1 week, and 22% later than 10 days respectively which deeply contrasted with the post fortification cohort where 7.5% had the MMC defect repaired in less than 8 h, 12.2% from 8 to 12 h, 66.5% 12-24 h, and 12.7% from 24 to 48 h and 1% later than 48 h, respectively (P < 0.01). Regarding the post fortification myelomeningocele anatomic and functional characteristics, defect size was measured as less than 3 cm in 7% of cases, 3 to 5 cm in 50% of cases, 5 to 7 cm in 42% of cases, and greater than 7 cm in 1% of cases. Thirteen percent of the cases had paraplegia due to a thoracic level, 10% had a high lumbar level, 58% had a middle lumbar level, 13% a lower lumbar level, and 6% only sacral compromise. When the data from the pre and post fortification cohorts were adjusted and compared, there was a reduction from thoracic/high lumbar cases from 26 to 23% (P < 0.56), with an increase of middle lumbar cases from 43 to 58% and a reduction from 25 to 13% of low lumbar cases (P < 0.01) while there was no change in the 6% percentage of sacral cases respectively. Lesions that were considered too extensive or larger than 7 cm decreased from 7 to 1% while associated kyphotic deformities decreased from 6 to 1.4% (P < 0.01); symptomatic Chiari II malformation at birth also decreased from 7 to 2% in the pre- and post-fortification cohorts respectively with all these changes being statistically significant (P < 0.01). Seventy-nine percent and 80% of the pre and post FAF cohorts required CSF VP shunting with a mean time for insertion of 10 days after spinal defect closure with no significant statistical change between the two groups. CONCLUSIONS: This study describes a four-staple folate fortified population of live-born patients with myelomeningocele lesions whose neurological level, defect size, and associated deformities such as spinal kyphosis and symptomatic Chiari II at birth suggest that folate fortification could have diminished the severity of this congenital disease.

WITHDRAWN: The Role of Neurosurgeons in Global Public Health: The Case of Folic Acid Fortification of Staple Foods to Prevent Spina Bifida.

The Publisher regrets that this article is an accidental duplication of an article that has already been published, http://doi.org/10.1016/j.wneu.2023.02.013. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/policies/article-withdrawal.

The role of neurosurgeons in global public health: the case of folic acid fortification of staple foods to prevent spina bifida.

OBJECTIVE: The global neurosurgery movement arose at the crossroads of unmet neurosurgical needs and public health to address the global burden of neurosurgical disease. The case of folic acid fortification (FAF) of staple foods for the prevention of spina bifida and anencephaly (SBA) represents an example of a new neurosurgical paradigm focused on public health intervention in addition to the treatment of individual cases. The Global Alliance for the Prevention of Spina Bifida-F (GAPSBiF), a multidisciplinary coalition of neurosurgeons, pediatricians, geneticists, epidemiologists, food scientists, and fortification policy experts, was formed to advocate for FAF of staple foods worldwide. This paper serves as a review of the work of GAPSBiF thus far in advocating for universal FAF of commonly consumed staple foods to equitably prevent SBA caused by folic acid insufficiency. METHODS: A narrative review was performed using the PubMed and Google Scholar databases. RESULTS: In this review, the authors describe the impact of SBA on patients, caregivers, and health systems, as well as characterize the multifaceted requirements for proper spina bifida care, including multidisciplinary clinics and the transition of care, while highlighting the role of neurosurgeons. Then they discuss prevention policy approaches, including supplementation, fortification, and hybrid efforts with folic acid. Next, they use the example of FAF of staple foods as a model for neurosurgeons' involvement in global public health through clinical practice, research, education and training, and advocacy. Last, they describe mechanisms for involvement in the above initiatives as a potential academic tenure track, including institutional partnerships, organized neurosurgery, neurosurgical expert groups, nongovernmental organizations, national or international governments, and multidisciplinary coalitions. CONCLUSIONS: The role of neurosurgeons in caring for children with spina bifida extends beyond treating patients in clinical practice and includes research, education and training, and advocacy initiatives to promote context-specific, evidence-based initiatives to public health problems. Promoting and championing FAF serves as an example of the far-reaching, impactful role that neurosurgeons worldwide may play at the intersection of neurosurgery and public health.

Preventing birth defects, saving lives, and promoting health equity: an urgent call to action for universal mandatory food fortification with folic acid.

July 20, 2021 marked the 30th anniversary of the publication of the landmark trial by the British Medical Research Council showing unequivocally that maternal intake of folic acid (vitamin B9) starting before pregnancy prevents most cases of infant spina bifida and anencephaly-two major neural tube defects that are severe, disabling, and often fatal. Mandatory food fortification with folic acid is a safe, cost-effective, and sustainable intervention to prevent spina bifida and anencephaly. Yet few countries implement fortification with folic acid; only a quarter of all preventable spina bifida and anencephaly cases worldwide are currently avoided by food fortification. We summarise scientific evidence supporting immediate, mandatory fortification with folic acid to prevent the development of spina bifida and anencephaly. We make an urgent call to action for the World Health Assembly to pass a resolution for universal mandatory folic acid fortification. Such a resolution could accelerate the slow pace of spina bifida and anencephaly prevention globally, and will assist countries to reach their 2030 Sustainable Development Goals on child mortality and health equity. The cost of inaction is profound, and disproportionately impacts susceptible populations in low-income and middle-income countries.

Craniopagus parasiticus: successful separation of a 28-week preterm newborn from parasite sibling twin bearing lethal congenital anomalies associated to Cantrell's pentad and sirenomelia-case-based review of the literature.

PURPOSE: This paper reviews the plausible etiological mechanisms, clinical features, preoperative analysis, and documented modern-day craniopagus parasiticus surgical separation attempts as well as an historical review of the few cases documented in the literature. METHODS: We describe the successful separation of a 28-week preterm newborn from its parasite sibling twin bearing lethal congenital anomalies associated to Cantrell's pentad and sirenomelia. Description of the case, plausible explanations on the mechanisms of conjointment along with the associated congenital abnormalities of the deceased twin are examined along with an historical revision of craniopagus parasiticus and their separation attempts with special attention to the previously undocumented attempt of the Dominican CP separation surgery by Lazareff et al. RESULTS: The use of the deceased twin cranial vault tissues (skin, bone, and duramater) as an autologous implant due to the identical genetical profile served to remodel and close the skull of the surviving twin with good esthetic results and no tissue rejection. To our knowledge, this is the youngest preterm set of craniopagus parasiticus separated in an emergency fashion with good functional and esthetic outcome. CONCLUSIONS: Craniopagus parasiticus is an infrequent subvariant of this rare form of twin conjointment which may require urgent separation due to the associated malformations of the parasitic twin; therefore, the fact that both siblings are genetically identical may prove as an advantage to use duramater, bone, and soft tissues from the parasitic twin as ideal grafts for covering the resultant defect after the separation has been performed.

Evaluation of the Pediatric Neuro-Oncology Resources Available in Chile.

PURPOSE: Pediatric neuro-oncology resources are mostly unknown in Chile. We report the human and material resources available in Chilean hospitals providing pediatric neuro-oncology services. METHODS: A cross-sectional survey was distributed to 17 hospitals providing pediatric neuro-oncology services (Programa Infantil Nacional de Drogas Antineoplásicas [PINDA] hospitals, 11; private, 6). RESULTS: Response rate was 71% (PINDA, 8; private, 4). Pediatric neuro-oncology services were mainly provided within general hospitals (67%). Registries for pediatric CNS tumors and chemotherapy-related toxicities were available in 100% and 67% of hospitals, respectively. CNS tumors were treated by pediatric oncologists in 92% of hospitals; none were formally trained in neuro-oncology. The most used treatment protocols were the national PINDA protocols. All WHO essential medicines for childhood cancer were available in more than 80% of the hospitals except for gemcitabine, oxaliplatin, paclitaxel, and procarbazine. The median number of pediatric neurosurgeons per hospital was two (range, 2-6). General neuroradiologists were available in 83% of the centers. Pathology specimens were sent to neuropathologists (58%), adult pathologists (25%), and pediatric pathologists (17%). Intensity-modulated radiotherapy, conformal radiotherapy, and cobalt radiotherapy were used by 67%, 58%, and 42% of hospitals, respectively. Only one private hospital performed autologous hematopoietic cell transplant for children with CNS tumors. CONCLUSION: A wide range of up-to-date treatment modalities are available for children with CNS tumors. Our survey highlights future directions to improve the pediatric neuro-oncology services available in Chile such as the expansion of multidisciplinary clinics, palliative care services, long-term cancer survivorship programs, dedicated clinical research support teams, establishing standardized mechanism for sending pathologic specimen for second opinion to international specialized centers, and establishing specialized neuro-oncology training program.

Global trends in the evaluation and management of cerebrospinal fluid shunt infection: a cooperative ISPN survey.

INTRODUCTION: Ventriculoperitoneal shunts (VPS) is commonly performed by pediatric neurosurgeons and there is no consensus in management of VPS infection as it relates to diagnosis and treatment. OBJECTIVE: We utilized an international practitioner-based survey to study the variability in VPS infection diagnostic and therapeutic measures. METHODS: A survey gauging practice patterns of pediatric neurosurgeons regarding VPS and its complication management was distributed. Survey endpoints were analyzed by VPS case volume and pediatric-focused case volume regarding diagnostic measures, use of cerebrospinal fluid (CSF) profile, microbiology, and treatment. RESULTS: A total of 439 surveys were distributed, with a response rate of 31%. Responders ranged from Americas (44.9%), European (31.4%), Asian (18.6%), African (2.5%), to Australian continents (2.5%). Practitioners were stratified based on number and percentage pediatric VPS performed. Institutions performing highest VPS and percentage pediatric case volumes had lower rate of VPS infection. Shunt tap was the most widely used diagnostic study. Overall CSF profile did not affect decision making towards VPS internalization, except for leukocyte count ≤ 20 × 109/L. Practitioners utilized 3 negative cultures prior to VPS internalization. Discrepancies in surgical management were noted amongst centers with high versus low VPS volume and proportion of pediatric-focused case volume. Practice patterns were not noted to be organism dependent. Antibiotic-impregnated shunts were utilized in the Americas and Europe over other regions but only in one third of all initial VPS or as a preventive strategy after a VPS infection has been resolved respectively. DISCUSSION: Survey results from 6 continents in VPS management revealed patterns of lower infection in high-volume centers, 3 negative cultures prior to internalization and aggressive surgical VPS infection management in high-volume institutions.

Fungal infections in pediatric neurosurgery.

INTRODUCTION: Invasive mycosis of the central nervous system represent a diverse group of diseases that have gradually emerged as not only opportunistic infections in patients with immune susceptibility due to congenital and acquired deficiency, immunomodulation, solid organ and stem cell transplantation, hematological malignancies, and chronic steroid use but also in selected risk populations such as low weight preterm infants, patients with shunted hydrocephalus and external ventricular drainages, skull base surgery, and head injury. OBJECTIVES: The purpose of this review is to familiarize the pediatric neurosurgeon with the most common mycosis and their clinical scenarios which can be encountered in the clinical practice, with special emphasis on clinical, radiological, and laboratory diagnosis beyond classical microorganism cultures as well as options in medical and surgical treatment given the high incidence of morbidity and mortality associated with these challenging entities. METHODS: We conducted an online database review (Ovid, PubMed) gathering relevant English language literature published in the last 20 years with special emphasis on recent breakthroughs in the diagnosis and treatment of invasive mycosis of the CNS as well as reported cases within the pediatric neurosurgical literature and their surgical management. RESULTS: Fungal agents capable of invading the CNS can behave as aggressive entities with rapid progression manifesting as overwhelming meningoencephalitis with vascular compromise or can lead to space-occupying lesions with abscess formation which require prompt diagnosis by either laboratory identification of the components of these biological agents and their host response or by obtaining tissue specimens for microbiological identification which may not be straightforward due to prolonged culture time. CONCLUSION: Following a high degree of suspicion with prompt initiation of antifungal agents and reversal of potential immunosuppressant therapies along with neurosurgical evacuation of intracranial collections or removal of infected hardware (CSF shunts) can lead to more optimistic outcomes of these complex clinical scenarios.

Neurocutaneous melanosis with associated Dandy-Walker complex.

CASE REPORT: The authors report the case of a child with neurocutaneous melanosis associated with a Dandy-Walker complex. Magnetic resonance (MR) images showed shortened T1-weighted images in areas involving the amygdala, mesencephalon, rostral brain stem, and superior cerebellar surface compatible with melanin deposits. There was also partial agenesis of the cerebellar vermis with an enlarged fourth ventricle cyst along with a high-lying torcular and ventricular enlargement. Endoscopic fenestration and biopsy of the cyst wall was performed without evidence of abnormal melanin deposits in the meninges. OUTCOME: The patient eventually required ventriculoperitoneal shunting and at 1-year follow-up did not develop evidence of primary CNS melanoma. ILLUSTRATION: Computed tomography and MR images consistent with neurocutaneous melanosis and the Dandy-Walker complex are presented along with photographs of the cutaneous nevi. DISCUSSION: The major clinical and radiological features of this rare association, with only 11 previously reported cases, are discussed in detail.

Intracavitary therapeutic options in the management of cystic craniopharyngioma.

BACKGROUND: Craniopharyngioma remains an entity of controversial management due to its critical anatomical relationships and its well-known recurrence tendency. Total resection, although desirable, is not always accomplished, and it is associated with potentially permanent endocrinological and visual deficits. Consequently, this situation has led to the development of new management strategies seeking lesser morbidity. Because a high percentage of these lesions are cystic, bleomycin and radioactive-emitting sources have been postulated as new therapeutic options with promising results. DISCUSSION: The information known to date regarding these substances is summarized and we perform a retrospective review of the clinical trials conducted over the past 30 years with special emphasis on outcome, morbidity, and mortality. Two cases from our institution are presented as examples of multimodality management. CONCLUSION: Overall, these newer options stand out as valid alternatives in the controversial management of these challenging tumors.

Craniopharyngioma and Cushing disease: case report.

Craniopharyngioma is a common sellar region tumor occurring in children. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. Patients with craniopharyngioma commonly present with visual deficits and hydrocephalus. The authors present the case of a child who presented with short stature and clinical evidence of Cushing disease (CD) associated with a suprasellar tumor. The patient underwent insertion of an Ommaya reservoir into the tumor's cystic portion. High adrenocorticotropic hormone (ACTH) levels were demonstrated within the cyst's fluid and in the serum. After adequate decompression of the tumor, the patient underwent total resection. The tumor pathology was compatible with an adamantinomatous craniopharyngioma and immunohistochemical studies failed to show staining for ACTH. Panhypopituitarism developed postoperatively in the patient and he received hormone substitution therapy with final adequate height and normal-high weight. The neurosurgical implications of CD along with a possible mechanism for this patient's presentation are discussed in detail on the basis of the pertinent literature.