Pediatric Neurosurgery最新文献

{"title":"Evaluation of the Endoscopic Third Ventriculostomy Success Score for Pediatric Hydrocephalus: Experience from a Singapore Children's Hospital.","authors":"Jensen Ang, Felicia H Z Chua, Sharmila Devi, David C Y Low, Wan Tew Seow, Sharon Y Y Low","doi":"10.1159/000546994","DOIUrl":"10.1159/000546994","url":null,"abstract":"<p><strong>Introduction: </strong>Endoscopic third ventriculostomy (ETV) is a well-established neurosurgical procedure. Concurrently, the Endoscopic Third Ventriculostomy Success Score (ETVSS) is a recognized validation tool commonly used to prognosticate the efficacy of this intervention. The main aims of this study are to review our institutional experience with ETV for pediatric hydrocephalus and evaluate its correlation with the ETVSS. Secondary aims include identification of other factors that are not part of the existing ETVSS and to corroborate our findings with contemporary literature.</p><p><strong>Methods: </strong>This is a single-institution, retrospective study. Patients under 19 years old who underwent ETV were included. Variables of interest such as patient characteristics, hydrocephalus etiology, procedural details, perioperative complications, neuroimaging features, and outcomes were collected. Radiological parameters curated from the literature such as third ventricular floor bowing, lamina terminalis bowing, third ventricular morphology index, and presence of prepontine adhesions (PPAs) are also included. For this study, the primary outcome measure is \"ETV success,\" defined as no need for shunt insertion to divert CSF at any point in time after ETV. Subsequent outcome of each ETV is correlated with the ETVSS. Additional factors are also independently assessed for their impact on the ETVSS in our study cohort.</p><p><strong>Results: </strong>Sixty-nine ETV cases were recruited for this study whereby ETV was successful in 63.8% (n = 44) cases. At 12 months' follow-up, their ETV stomas remained patent. Of note, 24.6% (n = 17) ETV failures occurred within 30 days of the procedure. In our series, ETV success correlated well with ETVSS. The ETV success rate was 0% for post-infectious and post-hemorrhagic etiologies. For the cases of ETV failure, definitive CSF diversion procedures were necessary within 3 months from their initial ETV. Logistic regression analysis showed ETVSS (odds ratio 1.068, p = 0.037) and the presence of PPA (p = 0.02) significantly correlated with ETV success.</p><p><strong>Conclusion: </strong>Our institutional experience in the use of ETV for pediatric hydrocephalus corroborates with findings from contemporary literature. In the context of our study, the ETVSS is applicable and the absence of PPA on neuroimaging demonstrates good correlation with ETV success.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"64-73"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369557","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":"Subtotal Resection with Proton-Beam Radiotherapy for Treatment of Pineal Parenchymal Tumor of Intermediate Differentiation in a Pediatric Patient.","authors":"Daniel E Fulkerson, Abigail Heck, Natalie Hauser, Daniel H Fulkerson","doi":"10.1159/000545882","DOIUrl":"10.1159/000545882","url":null,"abstract":"<p><strong>Introduction: </strong>Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are rare, pinealocyte-derived brain tumors that occur primarily in adults. The clinical prognosis fits somewhere between the benign pineocytoma and highly malignant pineoblastoma. There is very little published literature regarding this tumor in children and the existing pediatric information is enfolded with adult data in single-center reviews. The most common treatment recommendation for adults is aggressive resection, possibly followed by craniospinal irradiation (CSI) and/or chemotherapy. However, the adult literature is inconsistent, often contradictory, and does not address specific considerations in pediatric patients. To our knowledge, there are no papers specifically addressing the management and clinical considerations of PPTID in pediatric patients. As such, the optimal treatment strategy in children is unknown.</p><p><strong>Case presentation: </strong>We describe the treatment of a 6-year-old child who presented with obstructive hydrocephalus from a PPTID. The child was treated with a partial tumor resection followed by localized proton beam radiation. He has been followed for 8 years. Clinically, he is doing well, and his most recent MRI shows negligible residual tumor with no sign of recurrence.</p><p><strong>Conclusions: </strong>Our case suggests safe resection followed by proton beam radiotherapy may be effective in treating children with this exceedingly rare entity. While further study is needed, this strategy may avoid unnecessary surgical risk and the consequences of CSI on the developing pediatric nervous system.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"32-37"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144058559","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":"Neuroendoscopic Surgical Treatment of Cerebellar Vermis Tumors in Pediatric Patients: A Case Series.","authors":"Yu Zeng, Fang Liu, Zhuo Chen, Xiaohua Zhang, Sheng Zhao, Jian Liu, Chao Wang","doi":"10.1159/000545466","DOIUrl":"10.1159/000545466","url":null,"abstract":"<p><strong>Introduction: </strong>Neuroendoscopy is gaining traction as a minimally invasive technique for the resection of cerebellar vermis tumors. This study investigates the effectiveness and clinical experience of neuroendoscopic surgery for cerebellar vermis tumor resection.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on the clinical data of 18 patients with cerebellar vermis tumors treated using the German STORZ neuroendoscope between January 2021 and January 2024 at the Department of Neurosurgery, Guizhou Provincial People's Hospital, and Guizhou Hospital of Shanghai Children's Center. The surgical outcomes, pathological diagnoses, postoperative complications, and follow-up results were analyzed.</p><p><strong>Results: </strong>Of the 18 patients, total resection was achieved in 16 cases, subtotal resection in 1 case, and biopsy in 1 case. Postoperative pathology revealed 9 cases of medulloblastoma, 3 cases of ependymoma, and 5 cases of astrocytoma (2 WHO grade I, 2 grade II, and 1 grade III), along with 1 benign cerebellar lesion. Postoperative complications included malignant arrhythmia (1 case), cerebellar mutism (4 cases), and ataxia (13 cases). During the 1-36 months of follow-up, 2 patients developed communicating hydrocephalus, which improved following ventriculoperitoneal shunt placement. Recovery of cerebellar mutism occurred within an average of 21 days, while ataxia improved on average within 50 days. Tumor recurrence was observed in 3 patients.</p><p><strong>Conclusion: </strong>Neuroendoscopic resection of cerebellar vermis tumors, performed by skilled operators, provides effective surgical exposure and offers a viable alternative to traditional microscopy with satisfactory clinical outcomes.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"17-24"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712177","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 Electroencephalography Monitoring in the Child with a Bleeding Disorder: Challenges and Considerations.","authors":"Georgia Wong, Stephanie Gurevich, Saige Teti, Michael F Guerrera, Tesfaye Zelleke, William D Gaillard, Chima O Oluigbo","doi":"10.1159/000543194","DOIUrl":"10.1159/000543194","url":null,"abstract":"<p><strong>Introduction: </strong>Hereditary bleeding disorders stem from the absence or insufficient levels of particular clotting proteins, essential for facilitating coagulation in the clotting cascade. Among the most prevalent are hemophilia A (deficiency of factor VIII), hemophilia B (deficiency of factor IX), and von Willebrand disease (VWD). Management of pharmacoresistant epilepsy is more difficult in a patient with bleeding disorder due to increased risk of bleeding during surgery. While patients who have both a bleeding disorder and epilepsy are rare, reporting on the management of these patients who may require intracranial monitoring for pharmacoresistant epilepsy offers valuable insights into the challenges and considerations necessary for safely navigating the complex intersection of bleeding risk and seizure control.</p><p><strong>Case presentation: </strong>Two patients with bleeding disorders (VWD and factor XI deficiency) underwent invasive intracranial monitoring for medical refractory epilepsy followed by epilepsy focus resection surgery. Both patients were found to have a bleeding disorder during their preoperative laboratory work. After abnormal laboratories were reported, both patients were referred to hematology for further evaluation and surgical planning. The first patient was a 10-year-old boy with medically refractory focal epilepsy who was found to have type IIM VWD. He underwent surgery for subdural grid placement followed by resection on postoperative day 6. He required wilate® (human von Willebrand factor/coagulation factor VIII complex) infusions from the day of surgery prior to surgery through postoperative day 14. The second case was a 2-year-old boy with a history of tuberous sclerosis and medically refractory epilepsy who was found to have factor XI deficiency (hemophilia C) who required fresh frozen plasma and platelet transfusions throughout his hospitalization. He underwent surgery for sEEG followed by resection of the tubers. Both patients remained stable throughout their invasive monitoring and completed epilepsy resection surgeries without reported complications. Both patients achieved seizure freedom after surgery since their most recent follow-up of 1 month and 13 months.</p><p><strong>Conclusion: </strong>The two patients successfully underwent invasive neuromonitoring with subdural grids and sEEG for seizure focus identification followed by resective epilepsy surgery without bleeding complications while achieving seizure freedom. While epilepsy patients with a bleeding disorder should not automatically be denied surgery due to the increased risk of hemorrhage, it is crucial that any decision is based on a comprehensive, multidisciplinary evaluation. This case report highlights the potential for future meta-analysis and further conversations regarding improved protocols for patients with bleeding disorders.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"38-44"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142911121","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":"Novel Application of Connectomics to the Surgical Management of Pediatric Arteriovenous Malformations.","authors":"Shoaib A Syed, Fawaz Al-Mufti, Simon J Hanft, Chirag D Gandhi, Jared M Pisapia","doi":"10.1159/000547100","DOIUrl":"10.1159/000547100","url":null,"abstract":"<p><strong>Introduction: </strong>The emergence of connectomics in neurosurgery has allowed for construction of detailed maps of white matter connections, incorporating both structural and functional connectivity patterns. The advantage of mapping cerebral vascular lesions to guide surgical approach shows great potential. We aim to identify the clinical utility of connectomics for the surgical treatment of pediatric arteriovenous malformations (AVMs).</p><p><strong>Case presentation: </strong>We present two illustrative cases of the application of connectomics to the management of cerebral AVM in a 9-year-old and 8-year-old female. Using magnetic resonance anatomic and diffusion tensor imaging, a machine learning algorithm generated patient-specific representations of the corticospinal tract for the first patient, and the optic radiations for the second patient. The default mode network and language network were also examined for each patient. The imaging output served as an adjunct to guide operative decision-making. It assisted with selection of the superior parietal lobule as the operative corridor for the first case. Furthermore, it alerted the surgeon to white matter tracts in close proximity to the AVM nidus during resection. Finally, it aided in risk versus benefit analysis regarding treatment approach, such as craniotomy for resection for the first patient versus radiosurgery for the second patient. Both patients had favorable neurologic outcomes at the available follow-up period.</p><p><strong>Conclusion: </strong>Use of the software integrated well with clinical workflow. The output was used for planning and overlaid on the intraoperative neuro-navigation system. It improved visualization of eloquent regions, especially those networks not visible on standard anatomic imaging. Future studies will focus on expanding the cohort, conducting in pre- and postoperative connectomic analysis with correlation to clinical outcome measures, and incorporating functional magnetic resonance imaging.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"95-106"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144499343","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":"Infradentate Approach to the Fourth Ventricle with Tubular Retraction System for Medulloblastoma: Feasibility of a Minimally Invasive Technique to Avoid Anatomical Complications in a Pediatric Patient.","authors":"J Javier Cuéllar-Hernández, Omar R Ortega-Ruiz, Luis Alejandro Pérez-Ruano, Paulo Tabera-Tarello, Javier Terrazo-Lluch","doi":"10.1159/000545010","DOIUrl":"10.1159/000545010","url":null,"abstract":"<p><strong>Introduction: </strong>Medulloblastoma is the most common malignant tumor in the pediatric population. Current treatment of these lesions includes maximum safe resection, chemotherapy, and radiotherapy. Quality of life for these patients as well as postoperative complications remains with poorer prognosis compared to other posterior fossa tumors. Therefore, the surgical approach should be tailored to diminish the probability of coursing with postoperative complications such as cerebellar mutism. Minimally invasive techniques with tubular retractors have been described for supratentorial lesions. However, evidence remains scarce for lesions in the posterior fossa. Similarly, there are no available reports of the use of these techniques in pediatric patients.</p><p><strong>Case presentation: </strong>We present the case of an 11-year-old girl with a medulloblastoma. An infradentate approach was elected as the course of action looking to reduce the risk of postoperative cerebellar mutism syndrome. In the 2-year follow-up, the patient remained asymptomatic with no cerebellar signs nor recurrence of the lesion.</p><p><strong>Conclusion: </strong>We present the first case of a medulloblastoma treated with tubular ports in our center looking to discuss its feasibility for the treatment of posterior fossa tumors in pediatric patients. To the best of our knowledge, this is the first report of transcranial ports used in a pediatric patient for the resection of a medulloblastoma. Infradentate tubular resection could be a feasible and reproducible approach in pediatric patients, reducing the risk of injury to anatomical structures and cerebellar mutism.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"45-50"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143544422","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":"Cyclic Hypertonia and Hypotonia Associated with Intrathecal Baclofen Pumps: Findings and Treatments.","authors":"Megan V Ryan, Sophia Blasco, Kaitlin E Olson, Kim Sawyer, Joyce Oleszek, Swetha Sundar, Scott LeBeau, C Corbett Wilkinson","doi":"10.1159/000546529","DOIUrl":"10.1159/000546529","url":null,"abstract":"<p><strong>Introduction: </strong>Intrathecal baclofen therapy and baclofen pumps can lead to complications, including recurrent cycles of alternating hypertonia and hypotonia. The causes and optimal treatments for this issue remain unclear. This study reviews presentations, radiologic and surgical findings, treatments, and outcomes in cases of cyclic hypertonia/hypotonia.</p><p><strong>Methods: </strong>We reviewed patients with baclofen pumps treated at our hospital from 1998 to 2024 who developed cyclic hypertonia/hypotonia. Data collected included patient sex, etiology and type of tone abnormality, age at pump placement, age and weight at symptom onset, infusion rate and type at onset, side port access and catheter dye study results, treatments, surgical findings, and outcomes.</p><p><strong>Results: </strong>We identified 15 cases in 14 patients (10 females, 71%; 4 males, 29%). Among 248 patients with baclofen pumps, 38% were female. Females were significantly more likely to develop cyclic hypertonia/hypotonia (p < 0.01). The mean age at pump implantation was 10.2 years, and the mean time from last pump surgery to symptom onset was 645 days. All patients received enteric baclofen initially; 14 also had pump rate adjustments. Three patients improved without surgery, and one died during a hypotonia episode. Side port access showed patent catheters in 7 of 8 cases, with 6 of these showing good intrathecal dye dispersion. Surgery was performed in 11 cases, including two surgeries in 6 cases. Nine patients had pump replacements; 8 also had catheter revisions. Surgical findings included catheter kinks in 4 cases (36% of surgical cases) and leaks in 5 cases (45%), with 4 leaks at the pump-connector catheter connection. Six of 11 partial revisions (55%) and all 5 complete catheter replacements (100%) resulted in symptom resolution without recurrence. Replacing the pump-connector segment resolved symptoms without recurrence in 4 of 5 patients with leaks at this site. Overall, all patients undergoing surgery experienced symptom improvement without recurrence after one or two revisions.</p><p><strong>Conclusion: </strong>Adjusting pump rates and adding enteric baclofen may help some patients with cyclic hypertonia/hypotonia. If a leak at the pump-connector catheter segment is identified, segment replacement should be considered. When partial revisions fail, complete catheter replacement appears more effective, especially as a second surgery. Cyclic hypertonia/hypotonia is a treatable complication of baclofen pumps.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"51-63"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144152810","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":"Better Understanding the Orthopaedic Burden of Neurosurgical Intervention for Drug-Resistant Epilepsy in Paediatric Patients.","authors":"Adrian J Lin, Nakul S Talathi, Nicholas Gajewski, Rachel M Thompson","doi":"10.1159/000545112","DOIUrl":"10.1159/000545112","url":null,"abstract":"<p><strong>Introduction: </strong>Hemispherectomies, hemispherotomies, and lobectomies of the brain are neurosurgical techniques used to treat drug-resistant epilepsy (DRE). While effective for seizure control, these neurosurgical interventions can produce significant functional deficits including hemiparesis and iatrogenic cerebral palsy. In this study, we aimed to evaluate the resulting MSK pathology following surgery for DRE so that we may establish the incidence of new MSK pathology, identify risk factors for developing MSK pathology, and guide orthopaedic follow-up care.</p><p><strong>Methods: </strong>A retrospective chart review of 168 paediatric patients who underwent a brain hemispherectomy, hemispherotomy, or lobectomy between 2009 and 2018 was performed. Data including pre-existing neurological and orthopaedic conditions, presence of MSK pathology that emerged post-neurosurgical procedure, documented referral to orthopaedics, and post-operative interventions were collected. A multivariate logistic regression model was used to correlate predictive variables with the risk for developing new MSK pathology.</p><p><strong>Results: </strong>Of the 168 patients included, 45.2% (n = 76) developed a new MSK condition post-operatively. Of those with new MSK pathology, 30.3% (23) received orthopaedic follow-up. Of those, 34.8% (8) underwent a subsequent orthopaedic surgery. The median time to diagnosis of emerging MSK pathology following neurosurgical intervention was 0.7 months (range: 0-128 months), while the median time from emergence of symptoms to orthopaedic follow-up was 9.5 months (range: 2-161 months). Of the 28 patients who had MSK pathology prior to neurosurgical intervention, 42.8% (n = 12) were seen by orthopaedic providers following neurosurgery, of which 58.3% (n = 7) required orthopaedic surgery. Older age at the time of initial neurological surgery was significantly associated with decreased risk for developing new post-operative MSK pathology (OR 0.985, 95% CI: 0.979-0.911, p < 0.001), while repeat or revision neurosurgery was associated with increased risk (OR 3.728, 95% CI: 1.530-9.083, p < 0.01).</p><p><strong>Conclusion: </strong>Paediatric patients who undergo hemispherectomies, hemispherotomies, or lobectomies for DRE are subject to a significant post-operative burden of MSK disease, yet less than 1/3 of newly-affected patients receive orthopaedic follow-up - highlighting a gap between the need for and provision of orthopaedic care in this population.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"9-16"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588293","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":"Primary Gamma Knife Radiosurgery as a Treatment Option for Hamartoma of Floor of Fourth Ventricle: A Case Report of Pediatric Hemifacial Spasm.","authors":"Onam Verma, Manjul Tripathi, Adnan Hussain Shahid, Chirag Ahuja, Narendra Kumar, Arushi Gahlot Saini, Jitendra Kumar Sahu","doi":"10.1159/000543470","DOIUrl":"10.1159/000543470","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric hemifacial spasm (HFS) is rare, presenting early in infancy, and often fraught with subsequent psychomotor and intellectual deficits. Fourth ventricular hamartoma (FVH) is a rare cause of HFS with only 5 cases reported in literature. While gamma knife radiosurgery (GKRS) has been used to treat hypothalamic hamartomas, this is the first case of FVH treated with primary GKRS.</p><p><strong>Case presentation: </strong>A two-year-old female presented with persistent episodes of HFSs and dystonic posturing with an early resistance to medication. Thorough radiological profiling of the fourth ventricular tumor is the suggested tentative diagnosis of FVH. The patient's guardians refused surgical intervention and gave consent for GKRS aware of the lack of literature on its use in FVH. She underwent frame-based GKRS covering a total target volume of 0.986 cc with 13 Gy@50% with Leksell Perfexion. The patient showed a phasic response to GKRS with remarkable seizure control at a 1.5-year follow-up.</p><p><strong>Conclusion: </strong>Previous studies have suggested that gelastic seizures of hypothalamic hamartoma are comparable to HFSs of FVH. Our case exemplifies another key similarity between the two, i.e., a near-congruent phasic response to GKRS. This hints at the underlying pathophysiology of HFS in similar pathologies and GKRS as a treatment option in select patients.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"25-31"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142959072","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":"Multimodality Approach to a Pediatric Craniopharyngioma with Mixed Histological Features.","authors":"Sharon Y Y Low, Sharmila Devi, Enrica E K Tan, Sze Jet Aw, Khurshid Z Merchant, Ngee Lek, Marielle V Fortier, Lee Ping Ng, Sharon Y Y Low","doi":"10.1159/000546046","DOIUrl":"10.1159/000546046","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric craniopharyngiomas (CPGs) are histologically benign but clinically complex tumors. Traditional mainstays of treatment include surgical resection and radiotherapy. Molecular insights report that children tend to have the adamantinomatous subtype that is driven by the CTNNB1 pathway, while papillary CPGs prevalent in the adult population are characterized by BRAFV600E mutations. Mixed histological subtypes are rare. We report an unusual case of a pediatric CPG with both histological subtypes and discuss the management strategies in corroboration with contemporary literature.</p><p><strong>Case presentation: </strong>A 11-year-old female presented with symptoms of panhypopituitarism, optic atrophy, and bitemporal hemianopia. Magnetic resonance imaging (MRI) of her brain demonstrated a lobulated cystic-solid sellar-suprasellar lesion. The patient had emergent stereotactic aspiration of the lesion and insertion of an Ommaya reservoir. Intraoperative cyst fluid cytology confirmed wet keratin nodules, characteristic of adamantinomatous CPG. Following that, intracystic interferon-alpha therapy was commenced with good response for approximately 7 months. However, tumor progression was noted on subsequent MRI scans, with difficulty aspirating from the Ommaya reservoir. In view of this, the patient underwent an uneventful transsphenoidal resection of the tumor. Histology reported a craniopharyngioma with mixed adamantinomatous and papillary features with BRAFV600E positivity. MRI scans performed 2 months after surgery showed tumor recurrence. Decision was made for a trial of a dabrafenib - a BRAF inhibitor. After commencement of dabrafenib monotherapy for 1 month, radiological evaluation showed good tumor response. At 24 months posttreatment, the patient was well with her tumor in remission. In addition, no treatment-related adverse side effects were observed.</p><p><strong>Conclusion: </strong>We report a unique case of pediatric craniopharyngioma with mixed histological features that were managed successfully via a multimodality approach. Emphases are on molecular profiling of the said lesion, minimizing permanent morbidity and maintenance of quality of life for a growing child. In the context of tumors with BRAFV600E mutations, the use of targeted monotherapy can be considered.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"85-94"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060352","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}