Nir Shimony, Sarah Weatherspoon, Emily Hanzlik, Raja B Khan, Giles William Robinson, Ulrich-Wilhelm Thomale, Asim Choudhri, Soniya N Pinto, Jason Chiang, Paul Klimo, James W Wheless, George Jallo
{"title":"State-of-the-Art Management of Children with Intractable Epilepsy and Brain Tumors.","authors":"Nir Shimony, Sarah Weatherspoon, Emily Hanzlik, Raja B Khan, Giles William Robinson, Ulrich-Wilhelm Thomale, Asim Choudhri, Soniya N Pinto, Jason Chiang, Paul Klimo, James W Wheless, George Jallo","doi":"10.1159/000551567","DOIUrl":"10.1159/000551567","url":null,"abstract":"<p><strong>Background: </strong>Epilepsy is a common and frequently debilitating manifestation of pediatric brain tumors, especially low-grade epilepsy-associated tumors (LEATs) such as gangliogliomas, DNETs, and PXAs. In many children, seizures are the presenting symptom and may evolve into intractable epilepsy, affecting neurocognitive development and quality of life. Understanding the relationship between tumor biology, epileptogenic networks, and clinical presentation is essential for guiding timely and effective management.</p><p><strong>Summary: </strong>This review synthesizes current evidence and expert consensus on the integrated management of tumor-related epilepsy in children. We describe the anatomical, histopathological, and molecular correlates of epileptogenicity in pediatric brain tumors, with particular attention to the distinct clinical behavior of LEATs. Diagnostic strategies, including advanced imaging, neurophysiology, and invasive monitoring, are reviewed in the context of localizing the seizure onset zone while preserving function. The review outlines the indications and strategies for early surgical intervention, emphasizing that seizure control should be viewed as a primary therapeutic goal rather than a secondary outcome of tumor resection. Surgical approaches are discussed in relation to epilepsy duration, tumor location, and electroclinical concordance. For early, well-localized cases, lesionectomy may be sufficient, while longstanding epilepsy or tumors near eloquent cortex often require epilepsy-directed resections guided by intracranial monitoring. The role of preoperative ancillary tests (such as MEG, TMS, and neuropsychology) and intraoperative techniques (including ECoG and IONM) is highlighted for improving safety and precision.</p><p><strong>Key messages: </strong>(i) Epilepsy is a significant contributor to morbidity in children with brain tumors, particularly LEATs. (ii) Multidisciplinary care is essential for seizure localization and for optimizing outcomes in children with tumor-related epilepsy. (iii) Early surgical intervention is associated with better seizure and cognitive outcomes. (iv) Delayed treatment increases the risk of secondary epileptogenesis and long-term developmental effects. (v) Individualized surgical planning should balance oncologic and epilepsy goals from the first clinical encounter. (vi) Ancillary diagnostic and intraoperative tools enhance safety and help achieve maximal functional preservation.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"67-85"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147476377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Modern Aspects of Invasive Epilepsy Monitoring: Utility for Seizure Localization and Therapeutic Decision-Making.","authors":"Rohini Coorg, Elaine S Seto","doi":"10.1159/000550184","DOIUrl":"10.1159/000550184","url":null,"abstract":"<p><strong>Background: </strong>Children with drug-resistant epilepsy often require invasive monitoring to guide surgical interventions.</p><p><strong>Summary: </strong>Both subdural monitoring and stereoelectroencephalography (sEEG) have benefits and limitations in identifying seizure onset and mapping brain function. sEEG allows for more anatomically diffuse sampling, including deep structures like the thalamus. Monitoring leads to a better understanding of epileptic networks and their proximity to eloquent cortex. This often guides the decision between resective/ablative therapies and neuromodulation. There is increasing interest in the use of thalamic stimulation in neuromodulation, although pediatric data are limited.</p><p><strong>Key message: </strong>The increase in invasive monitoring in the USA coupled with the emergence of additional surgical treatments offers new hope to children with refractory epilepsy.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"31-40"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13218702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145893528","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
David Bonda, Clayton P Mosher, Naga Sai Anagha Devulapalli, Ishan Kanungo, Edwina Tran, Stuart G Finder, Ueli Rutishauser, Adam Mamelak
{"title":"Single-Neuron Recordings Research in Children: Ethical Considerations, Feasibility, Technical Aspects, and Scientific Opportunities.","authors":"David Bonda, Clayton P Mosher, Naga Sai Anagha Devulapalli, Ishan Kanungo, Edwina Tran, Stuart G Finder, Ueli Rutishauser, Adam Mamelak","doi":"10.1159/000549937","DOIUrl":"10.1159/000549937","url":null,"abstract":"<p><strong>Background: </strong>Human intracranial recordings and single-neuron recordings in particular have provided much knowledge on the mechanisms of human cognition and its impairment by disease. Improvements in recording technology, experimental design, and computational analysis methods have permitted an increasingly sophisticated understanding of uniquely human brain processes, including those underlying executive function, memory, and language. Despite the routine clinical use of intracranial recordings for invasive epilepsy monitoring in the pediatric population, there remains a significant gap between the associated research conducted in adult and pediatric neuroscientific investigation.</p><p><strong>Summary: </strong>Single-neuron recordings in pediatric epilepsy patients are ethical, technically feasible, and safe. These data can provide mechanistic insights into the neurophysiology of the developing human brain.</p><p><strong>Key messages: </strong>Routine use of invasive electrophysiological monitoring via stereoelectroencephalography studies in pediatric drug-resistant epilepsy offers opportunities to extend the utility of single-neuron recordings to the pediatric population and advance our knowledge of the neuronal basis of behaviors in children.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"111-125"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12709666/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145688689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Barbara Buccilli, Amna Hussein, Khaled M Taghlabi, Monique Mitchell, Diogo Haddad Santos, Raphael Bertani, Wellingson Paiva, Renato Anghinah, Amir H Faraji
{"title":"Shunt Complications in Syndromic versus Non-Syndromic Pediatric Hydrocephalus: A Propensity-Matched Multicenter Analysis of 35,234 Patients.","authors":"Barbara Buccilli, Amna Hussein, Khaled M Taghlabi, Monique Mitchell, Diogo Haddad Santos, Raphael Bertani, Wellingson Paiva, Renato Anghinah, Amir H Faraji","doi":"10.1159/000550188","DOIUrl":"10.1159/000550188","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric hydrocephalus is associated with congenital malformation syndromes, which may alter cerebrospinal fluid dynamics and increase the complexity of surgical management. We aimed to compare rates of shunt placement, reprogramming, revision, removal, and dysfunction in pediatric patients with hydrocephalus, with and without congenital syndromes.</p><p><strong>Methods: </strong>We conducted a retrospective analysis using TriNetX, identifying pediatric patients with hydrocephalus. Cohort 1 included patients with congenital syndromes (N = 17,617). Cohort 2 included matched patients without syndromes (N = 17,617). Propensity score matching was performed across demographic and clinical variables. Outcomes included shunt placement, reprogramming, revision, removal, and dysfunction. Risk measures were calculated at 1-year follow-up.</p><p><strong>Results: </strong>Shunt placement was similar between cohorts. However, syndromic patients had significantly higher rates of reprogramming (10.0% vs. 4.2%; odds ratio [OR]: 2.52; p < 0.001), revision (9.3% vs. 3.3%; OR: 3.03; p < 0.001), removal (2.0% vs. 0.9%; OR: 2.24; p < 0.001), and dysfunction (23.5% vs. 10.9%; OR: 2.52; p < 0.001). Intervention-free survival was lower in syndromic patients for all outcomes except initial shunt placement. Across single congenital syndromes, patients with hydrocephalus demonstrated elevated rates of shunt-related complications compared to matched controls. In conditions like Arnold-Chiari and encephalocele, the rates of shunt dysfunction exceeded 30%, with OR above 3.0 and highly significant p values. Alport syndrome, arhinencephaly, and craniosynostosis showed elevated risks for reprogramming, revision, and dysfunction. Down syndrome demonstrated higher dysfunction rates (27.1% vs. 14.3%; OR: 2.22). Despite identical shunt placement rates, the downstream need for surgical intervention diverged sharply, highlighting high-risk phenotypes.</p><p><strong>Conclusion: </strong>Pediatric hydrocephalus associated with congenital syndromes carries a markedly higher risk of shunt-related complications. These findings support the need for clinical surveillance and individualized care strategies in this group.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"137-153"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13245943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145812193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Palliative Nature of Pediatric Epilepsy Surgery and Our Procedures.","authors":"Hunter S Futch, Henry M Skelton, Nealen G Laxpati","doi":"10.1159/000549249","DOIUrl":"10.1159/000549249","url":null,"abstract":"<p><strong>Background: </strong>Destructive surgical treatment for drug-resistant epilepsy may lead to complete seizure relief. In cases where a surgical cure is unavailable, epilepsy surgery can still alleviate seizure burden by decreasing reliance on medication, decreasing the severity or frequency of seizures, or decreasing the frequency of status epilepticus.</p><p><strong>Summary: </strong>In this article, we discuss the role of palliative epilepsy surgical techniques in pediatric patients, relevant syndromes characterized by refractory epilepsy, and pertinent procedures and considerations. We review both destructive and neuromodulatory therapies, the current evidence supporting their use, and discuss future indications and expansion of techniques.</p><p><strong>Key message: </strong>Our expanding epilepsy surgery armamentarium has the potential to provide significant palliative therapy for our pediatric patients and significantly improve their quality of life.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"5-15"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13218696/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145454051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Development, Content Validity, and Test-Retest Reliability of a Childhood Hydrocephalus Severity Scale.","authors":"Olufemi Emmanuel Idowu, Jeuel Ogooluwa Idowu","doi":"10.1159/000544165","DOIUrl":"10.1159/000544165","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to develop and validate a reliable, multidomain scale for assessing childhood hydrocephalus severity and to enhance communication, guide treatment decisions, and improve patient care.</p><p><strong>Methods: </strong>A stepwise consensus approach informed by a modified Delphi technique was employed. Healthcare professionals participated in anonymous surveys and face-to-face meetings to define the core domains of the scale. Content validity, internal consistency, and inter-rater reliability were assessed.</p><p><strong>Results: </strong>The Delphi process yielded a refined 7-item, 10-point Childhood Hydrocephalus Severity Scale (CHS) focusing on age, Evans index, associated malformations, neurological deficit, intraventricular hemorrhage, and mid-arm circumference. Content validity analysis using the Content Validity Index (CVI) demonstrated strong agreement (mean I-CVI = 0.91) among experts regarding the relevance of CHS items. All individual item CVI scores exceeded 0.8, supporting the inclusion of each factor. The CHS exhibited excellent internal consistency (Cronbach's alpha = 0.988). High intraclass correlation coefficients (ICCs) were observed for both single measures (ICC = 0.902, 95% CI: 0.862-0.931) and average measures (ICC = 0.985, 95% CI: 0.978-0.990), indicating near-perfect agreement between raters. Both ICC values were statistically significant (p < 0.001).</p><p><strong>Conclusion: </strong>The CHS demonstrates promising potential as a reliable and valid tool for childhood hydrocephalus severity assessment. This scale has the potential to enhance communication, guide treatment decisions, and improve patient care in childhood hydrocephalus.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"1-8"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143415716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yujia Shentu, Kriti Bhayana, Eliana E Bonfante-Mejia, Stuart M Fraser, David I Sandberg
{"title":"Internal Cerebral Vein Thrombus Mimicking Thalamic Neoplasm in a Child with Down Syndrome.","authors":"Yujia Shentu, Kriti Bhayana, Eliana E Bonfante-Mejia, Stuart M Fraser, David I Sandberg","doi":"10.1159/000548827","DOIUrl":"10.1159/000548827","url":null,"abstract":"<p><strong>Introduction: </strong>Thalamic strokes are uncommon in children and often arise from underlying conditions such as coagulopathy. Their clinical and radiological resemblance to neoplasms - with shared symptoms and overlapping MRI features - makes accurate diagnosis particularly challenging.</p><p><strong>Case presentation: </strong>A 2-year-old boy with trisomy 21 (Down syndrome) presented with 5 days of altered mentation and fussiness, nausea, vomiting, and seizures. CT head demonstrated a right thalamic edematous lesion. MRI showed a lesion with imaging features suggestive of a thalamic neoplasm that was ultimately found to be an infarct secondary to right internal cerebral vein thrombus. The patient was treated with heparin and returned to his clinical baseline.</p><p><strong>Conclusion: </strong>Given their predisposition to coagulopathy, patients with Down syndrome require meticulous imaging evaluation. CT venogram can be an essential part of workup for this patient population.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"114-119"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145240226","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Invasive Intracranial Pressure Monitoring for Pediatric Traumatic Brain Injury: Do Data Support Dogma?","authors":"Debajyoti Datta, Albert Tu","doi":"10.1159/000549399","DOIUrl":"10.1159/000549399","url":null,"abstract":"","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"153-155"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145688661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Contralateral Transmaxillary Approach for a 13-Year-Old Boy with a Petrous Apex Cholesterol Granuloma: A Case Report.","authors":"Yasuhiro Arai, Jun Suenaga, Mitsuru Sato, Daisuke Sano, Tetsuya Yamamoto, Nobuhiko Oridate","doi":"10.1159/000546531","DOIUrl":"10.1159/000546531","url":null,"abstract":"<p><p><p>Introduction: Surgical extirpation of a cholesterol granuloma in the petrous apex, located dorsal to the petrous part of the internal carotid artery (ICA), is challenging. Herein, we report a pediatric case of a cholesterol granuloma of the petrous apex treated using the endoscopic contralateral transmaxillary (CTM) approach. Case Presentation: A 13-year-old boy presented with a left-sided headache, slight hypoesthesia in the left V1 area, and severe neuralgia of the left auriculotemporal nerve. Magnetic resonance imaging (MRI) revealed a high-intensity mass without gadolinium enhancement. The patient's headache was unresponsive to various medications. After careful evaluation, an endoscopic CTM approach was selected for the extirpation of the granuloma. Postoperatively, the patient did not experience headache or associated neurological complications. MRI at 46 months revealed no recurrence. Conclusion: The endoscopic CTM approach can be used for excising cholesterol granulomas of the petrous apex located posterior to the petrous part of the ICA without causing severe complications. This approach can be considered useful for pediatric cases in which granulomas are not accessible via the transnasal endoscopic transsphenoidal approach. </p>.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"79-84"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12215168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144175854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Evaluation of Association of Prematurity with Benign Enlargement of Subarachnoid Space in Infants Referred for Macrocephaly.","authors":"Fardad T Afshari, Katie Herbert, Amy Drew, Joshua Pepper, Desiderio Rodrigues","doi":"10.1159/000547283","DOIUrl":"10.1159/000547283","url":null,"abstract":"<p><strong>Introduction: </strong>Benign enlargement of subarachnoid space (BESS) is one of the causes of macrocephaly in infants. The aetiology of this condition remains a subject of controversy, with poor cerebrospinal fluid absorption as the most commonly believed underlying theory. Prematurity has been suggested as possible risk factor; however, this is a topic of debate with paucity of data. In this study, we investigated association of prematurity with BESS in our cohort of patients.</p><p><strong>Methods: </strong>A retrospective review of patients seen in outpatient neurosurgical BESS clinic (period 2016-2023) was carried out. BESS was defined as enlarged frontal subarachnoid space diagnosed on CT or MRI brain obtained for macrocephaly. Demographics information including sex, age, prematurity/gestation at birth, grade of prematurity as well as any neurosurgical interventions were recorded. Prematurity was defined as per World Health Organization guidelines as birth at gestation<37 weeks. Grade of prematurity was defined as <28 weeks as extreme preterm, 28-32 weeks as severe preterm, 32-34 weeks as moderate preterm, and 34-37 weeks as late preterm.</p><p><strong>Results: </strong>Over the period of study, 100 children with BESS were seen in the outpatient clinic setting with mean age of 12.3 months (range 0.5-54 months) with M78:F22 ratio (M:F = 3.5:1). Overall, 19 out of 100 children with BESS were born premature (19%) with a mean age of 11.9 months at diagnosis. Premature group included 0% extreme preterm, 21% severe preterm, 15.8% moderate preterm, and 63.2% late preterm. Rate of prematurity at 19% in this cohort was 2.4-fold the rate of UK prematurity of 7.5-7.9% (p = 0.023). Eight out of 100 (8%) patients had concurrent subdural collection of which one belonged to premature group. All subdural collections were managed non-operatively. There was no association between prematurity and subdural formation. No child required cerebrospinal fluid diversion.</p><p><strong>Conclusion: </strong>BESS remains a poorly understood entity. Considering the baseline rate of live preterm births in UK at 7.5-7.9%, rate of prematurity in this cohort of children was significantly higher at 19%. This study may support that prematurity is a possible risk factor.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"74-78"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144576927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}