Spring-assisted cranioplasty is an established technique for the correction of isolated sagittal craniosynostosis, yet data on long-term clinical durability and patient-perceived outcomes remain limited. The purpose of this study was to evaluate long-term surgical, aesthetic, and patient-reported outcomes following spring-assisted cranioplasty, with a particular focus on the need for secondary cranial surgery and concordance between surgeon- and parent-reported assessments. A single-centre cohort study was performed, including consecutive patients with isolated, nonsyndromic sagittal synostosis treated with spring-assisted cranioplasty between April 2010 and September 2015. Patients were followed within a standardized multidisciplinary craniofacial pathway extending into late childhood and adolescence. Long-term outcomes were assessed using reoperation rates, Kaplan–Meier reoperation-free survival analysis, surgeon-reported Whitaker classification, and structured parent-reported satisfaction measures. Ninety-one of the original 100 patients had complete long-term follow-up and were included, with a mean follow-up duration of 10.0 years (range 5–15 years). Nine patients (9.9
The supraorbital keyhole approach via transciliary incision represents a minimally invasive surgical corridor that is increasingly applied in pediatric neurosurgery; however, large childhood-specific series remain scarce. This study reports our two-center experience with this approach in a heterogeneous pediatric population. A retrospective analysis was performed of all pediatric patients who underwent surgery via the supraorbital keyhole approach through a transciliary incision at Ankara University and Rome Catholic University between 2017 and 2024. Patient demographics, pathological diagnoses, extent of resection, complications, and clinical outcomes were recorded. The extent of resection was classified as gross total resection (GTR), subtotal resection (STR), or near-total resection (NTR) based on the postoperative MRI findings. Twenty-six patients (mean age, 8.7 years; range, 0.7–17 years) were included in the study. The pathologies included craniopharyngioma (n = 5), cavernous malformation (n = 4), optic pathway glioma (n = 3), pilocytic astrocytoma (n = 2), epidural hematoma (n = 2), frontal abscess (n = 2), and other lesions (n = 8). Among the 20 patients with discrete resectable lesions, GTR was achieved in 11 (55
Ectopic colloid cysts are rare, benign, congenital lesions derived from the endoderm. They are most commonly located in the anterior portion of the third ventricle but can occasionally arise in the sellar/suprasellar region [1, 7, 12]. We report a pediatric patient with an ectopic colloid cyst presenting with short stature and central diabetes insipidus-a location not previously documented in children. Endoscopic endonasal transsphenoidal resection achieved subtotal removal with preservation of the pituitary stalk, and the patient remained symptom-free at 21-month follow-up.
To explore the technical methods and advantages of prenatal diagnosis of fetal intraspinal lipomas. This was a prospective study. From August 2018 to August 2024, 315 fetuses suspected of having spinal neural tube malformations after conducting prenatal ultrasound were treated at the Neurosurgery Outpatient Department of the First Medical Center of the PLA General Hospital. Fetal magnetic resonance imaging (MRI) examinations were performed within 72 h after the ultrasound diagnosis. A total of 186 singleton pregnancies where fetuses have already been born were selected. MRI was performed within 1 month after birth. Using postnatal MRI as the gold standard, 58 fetuses diagnosed with intraspinal lipoma through postnatal MRI were selected. The diagnostic coincidence rates of prenatal ultrasound and fetal MRI in the prenatal diagnosis of intraspinal lipomas were analyzed. Prenatal ultrasound correctly diagnosed 35 cases (35/58, 60.3
Spring-mediated cranioplasty (SMC) is an established minimally invasive technique for the treatment of sagittal craniosynostosis. This study describes our surgical technique and evaluates long-term morphometric outcomes following SMC. A retrospective review was performed of subjects who underwent SMC as primary treatment of sagittal craniosynostosis at our institution between 2011 and 2019. Demographic variables, perioperative data, spring characteristics, and cranial index (CI) were obtained. Subjects were stratified by their use of post-operative helmeting. Longitudinal CI changes were evaluated using linear mixed-effects models. Differences in CI change between subjects receiving 2 vs 3 springs were compared using Mann–Whitney U tests. Linear regression was used to evaluate total spring force as a predictor of percent change in CI. Seventy subjects were included. Seventy-four percent (n = 52) of the cohort received springs treatment only, whereas 26
An 8-month-old infant that presented to our service with signs of increased intracranial pressure, seizures and radiographic findings of multiple intracranial cavernomas, most notably a left temporal cavernoma that had bled and was causing significant local mass effect. Outside the CNS, the infant had cutaneous manifestations of cavernous malformation syndromes. Surgical resection of the left temporal haemorrhage was pursued, and the infant recovered well. Subacute management involved ongoing clinical reviews, including repeat MRI scans, and regular propranolol use, aiming to reduce the incidence and burden of clinically significant haemorrhages, as well as levetiracetam for seizure control. Genetic testing confirmed the K-CRIT/CCM-1 gene mutation as the causative aetiology, with significance for this child and their immediate family. Furthermore, the lessons learned in this case illustrate the clinical findings and management considerations in an infant with intracranial haemorrhage secondary to a familial form of cavernous malformation syndrome.
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly malignant pediatric brain tumor with dismal prognosis. We aimed to characterize dynamic survival patterns using conditional survival (CS) and annual hazard rate (AHR) analyses, identify key prognostic factors through machine learning (ML)–based feature selection, and develop an interpretable CS-nomogram for individualized prognostication. Data of AT/RT patients diagnosed between 2000 and 2022 were obtained from the SEER database. CS and AHR analyses were performed to assess temporal survival dynamics. Four complementary algorithms—LASSO regression, Boruta algorithm, stepwise regression, and best subset regression—were used for feature selection. The final CS-nomogram was validated using ROC curves, calibration plots, and decision curve analysis (DCA). Model interpretability was evaluated with Shapley additive explanations (SHAP), and a web-based calculator was created for clinical application. A total of 382 patients were analyzed. The 5-year survival probability at diagnosis was 34.11
Cardiogenic brain abscesses (CBA) are rare infections that primarily affect children with cyanotic congenital heart disease (CCHD). Despite longstanding clinical awareness, gaps remain in our understanding of long-term outcomes and the actual recurrence risk. This study analyzed the clinical profile, management, and long-term outcomes of a large institutional cohort, with an emphasis on quantifying the recurrence risk. We retrospectively reviewed the records of 34 pediatric patients (aged < 15 years at index abscess diagnosis) who underwent CBA management at our institution between January 2001 and February 2025. Data on clinical presentation, microbiology, surgical intervention, and cardiac status were also collected. Long-term follow-up was conducted using clinical records and standardized telephone surveys. The mean age at presentation was 8.2 years. Streptococcus spp. was the most common isolate (15/34), and 44
Transcranial electrical stimulation motor evoked potential (TES-MEP) monitoring during pediatric spinal surgery is technically challenging due to developmental corticospinal physiology. We quantified the effects of electrode montage, stimulation pattern, and interstimulus interval (ISI) on motor threshold (MT) to define a practical optimization protocol for pediatric neuromonitoring. Eighty-one pediatric patients (0.5–17 years) undergoing spinal or spinal cord surgery were analyzed using a prospective within-patient design. Effects of montage and stimulation pattern were assessed with repeated-measures ANOVA. ISI modulation (2, 3, 4 ms) was evaluated within the linked quadripolar montage with double-train stimulation (LQP-DTS) in patients without floor effect (n = 65). Multivariable regression identified determinants of baseline MT, ISI responsiveness, and pulse escalation. Montage and stimulation pattern exerted large independent effects on MT (η2p = 0.986 and 0.969; both p < 0.001). LQP-DTS reduced mean MT by 77.8 V (95
Supratentorial pediatric ependymomas (ST-EPNs) represent a rare and biologically heterogeneous subgroup of central nervous system tumors. The 2021 WHO molecular classification has redefined their diagnostic framework, emphasizing prognostic and therapeutic implications. This study analyzes clinical, surgical, and molecular features of pediatric ST-EPNs treated at a single-institution, with particular focus on outcomes and prognostic variables. We retrospectively reviewed pediatric patients with histologically confirmed supratentorial ependymomas treated between 2007 and 2023. Tumors were reclassified according to the 2021 WHO molecular criteria. Clinical presentation, imaging characteristics, tumor location, molecular subtype, extent of resection, adjuvant therapies, recurrence patterns, and long-term outcomes were evaluated. Twelve patients were included (mean age 7.4 years). Two tumors were intraventricular and ten extraventricular. Molecular profiling identified ZFTA fusion-positivity in 11 cases and YAP1 fusion-positivity in one case. Gross total resection was achieved in 11 of 12 patients, whereas one patient underwent near-total resection because of vascular encasement of the middle cerebral artery, making complete resection unsafe. Intraventricular tumors presented earlier, required complex surgical management, and were associated with higher treatment burden. Extraventricular tumors commonly presented with seizures and cystic imaging features, with favorable postoperative recovery. Recurrence occurred in 3 patients, successfully managed with repeat surgery and adjuvant therapy. At a mean follow-up of 8 years, all patients were alive with good neurological outcomes. Secondary neoplasms were observed in isolated cases following multimodal treatment. This single-institution molecularly reclassified series highlights clinically relevant differences between intraventricular and extraventricular pediatric supratentorial ependymomas in presentation, surgical complexity, and treatment burden. Our findings support maximal safe resection as the central therapeutic strategy while emphasizing the value of integrated molecular diagnostics and prolonged surveillance, particularly in light of late recurrence and secondary neoplastic events.
BACKGROUND:Factor XIII (FXIII) deficiency is a rare autosomal recessive coagulation disorder characterized by impaired fibrin stabilization and delayed bleeding despite normal routine coagulation parameters. Intracranial hemorrhage represents its most severe and life-threatening manifestation, particularly in the infant population. CASE DESCRIPTION:We report a 5-month-old female infant presenting with a right frontal lesion following minor trauma, initially suggestive of tumor or abscess based on its radiological appearance. The patient underwent craniotomy; however, recurrent postoperative hematomas developed despite consistently normal routine coagulation tests. Further targeted hematological evaluation revealed markedly reduced FXIII activity (15%, reference range 70-140%), confirming the diagnosis of FXIII deficiency. Following initiation of replacement therapy with fresh frozen plasma, no further hemorrhagic episodes occurred. CONCLUSION:Factor XIII deficiency should be considered in infants presenting with recurrent intracranial hemorrhage and normal routine coagulation tests, particularly in the presence of clinical risk factors such as neonatal umbilical bleeding and parental consanguinity. Early targeted laboratory evaluation and prompt initiation of replacement therapy may prevent repeated surgical interventions and improve clinical outcomes.
Pineal region tumors pose significant surgical challenges due to their deep location and proximity to critical neurovascular structures. Despite multiple described techniques, no consensus exists on the optimal surgical approach, with the supracerebellar infratentorial (SCIT) and occipital interhemispheric transtentorial (OITA) approaches representing the most adopted corridors. A systematic literature review was conducted to evaluate surgical approaches for pineal region tumors and their associated complication profiles. In addition, anatomical dissections combined with a tumor simulation technique were used as illustrative tools to compare exposure patterns obtained through SCIT, SCIT with tentorial incision (SCIT + T), and OITA approaches. The systematic review, comprising 27 studies and 1774 patients, identified OITA (42.21
To evaluate clinical predictors of ventriculoperitoneal (VP) shunt malfunction in children presenting to the pediatric emergency department and to quantify cumulative imaging-related radiation exposure. This retrospective study included 84 patients aged 0–18 years with VP shunts who were followed between August 2014 and July 2018. A total of 123 shunt-related emergency department visits were analyzed regarding clinical presentation, physical examination findings, laboratory results, imaging utilization, and outcomes. Multivariable logistic regression was performed to identify independent clinical predictors of shunt malfunction. Model performance was assessed using receiver operating characteristic (ROC) analysis. Shunt malfunction was diagnosed in 39.8
Children with spinal dysraphism (SD), and particularly those with open spinal dysraphism (OSD; myelomeningocele/myeloschisis), have historically been classified as a uniquely high-risk population for latex allergy, with most published evidence derived from Western cohorts reporting prevalence rates of 26–70
Pediatric posterior fossa tumors represent a major subset of childhood central nervous system neoplasms; however, overlapping MRI features often hinder accurate non-invasive characterization. This study aimed to evaluate machine learning (ML) and deep learning (DL) models for classifying these tumors using MRI-derived radiomic features. This retrospective study analyzed MRI data from 63 pediatric patients with confirmed posterior fossa tumors, including 21 medulloblastoma (MB), 20 pilocytic astrocytoma (PA), 11 ependymoma (EP), and 11 diffuse midline glioma (DMG) cases. T2-weighted imaging, diffusion-weighted imaging, and apparent diffusion coefficient sequences showing the best single-model performance were used to construct gradient boosting machine (GBM), decision tree (DT), and random forest (RF) models and their ensemble combinations. Model performance was evaluated using standard classification metrics. A ResNet101V2-based DL model was developed using multiple MRI sequences. ML models were validated using fivefold cross-validation, whereas the DL model was trained using a 67/33 train–test split with data augmentation. The RF + GBM ensemble achieved the highest ML performance, with an overall accuracy of 78
Vertebrobasilar dolichoectasia (VBDE) is a rare condition characterized by elongation, dilation, and tortuosity of the vertebral and basilar arteries, typically seen in adults with hypertension or connective tissue disorders. Pediatric cases of VBDE are exceptionally rare, and its diagnosis and treatment in children present unique challenges. We report the case of a 14-year-old male who presented with a three-year history of intermittent headaches, slurred speech, and new-onset bilateral deafness, among other cranial nerve deficits. Both magnetic resonance arteriography (MRA) and digital subtraction angiography (DSA) revealed a significantly dilated and tortuous basilar artery, with compression of the brainstem and cerebellum, consistent with VBDE. The patient underwent a right vertebral artery (V3) to superior cerebellar artery (SCA) bypass with interposition grafting from the left radial artery and clip occlusion of the V3 of the right vertebral artery. Postoperatively, the patient experienced some improvement in his ataxia and facial palsy, though his bilateral deafness remained. This case is notable for the successful surgical intervention in a pediatric patient, demonstrating that early surgical management can potentially alleviate symptoms and slow disease progression in younger individuals. This report contributes to the limited literature on VBDE in pediatric patients, underscoring the importance of considering VBDE as a differential diagnosis in children with unexplained cranial nerve deficits or brainstem compression. The case also highlights the complexity of surgical management and the potential for positive outcomes in pediatric patients compared to adults, where the disease often carries a poor prognosis.
Magnetic resonance imaging (MRI) is routinely used to assess extent of resection after pediatric brain and spine tumor surgery and is commonly performed intraoperatively (ioMRI) or postoperatively (postopMRI). This study describes the implementation, feasibility, and workflow characteristics of end-of-surgery MRI (eosMRI), performed after wound closure but before termination of general anesthesia within a single-center pediatric neurosurgical practice and reports procedural and patient-related parameters observed over a 10-year period. We conducted a retrospective, single-center observational study including all pediatric brain and spine tumor surgeries performed with eosMRI, ioMRI, or postopMRI between July 2013 and September 2022. MRI modality selection followed institutional workflow patterns and surgeon judgment rather than predefined allocation criteria. Recorded variables included anesthesia time, surgery duration, imaging duration, patient age, tumor location, imaging-guided additional resection, and extent of resection (EOR). A total of 129 surgeries in 106 children (mean age 9 years, range 0.3–18) were analyzed: 45 eosMRI (35
Background Caudal duplication syndrome (CDS) is a rare congenital condition involving duplication of gastrointestinal, genitourinary, and distal spinal structures that originate from the cloaca. Lipomeningomyelocele (LMMC) with tethered cord represents an uncommon but surgically relevant manifestation within this heterogeneous spectrum. Case presentation A 3-year-old girl with CDS and persistent cloaca was referred for neurosurgical evaluation of a giant sacral LMMC and a low-lying conus medullaris consistent with tethered cord. Although neurologically intact at presentation, spinal magnetic resonance imaging demonstrated significant tethering associated with a large lipomatous lesion. The patient underwent prophylactic microsurgical detethering and lipoma resection under intraoperative neuromonitoring. A cerebrospinal fluid fistula developed 2 weeks postoperatively and was successfully repaired. At follow-up, she remained neurologically stable with preserved motor function. Literature review A focused review identified four previously reported cases of CDS associated with LMMC and radiologically confirmed tethered cord requiring surgical detethering. Cases lacking either LMMC or radiological evidence of tethering were not included in this subgroup, underscoring the importance of distinguishing isolated lipomas from true tethered cord pathology within the CDS spectrum. Conclusion CDS associated with LMMC and tethered cord represents a rare but clinically significant subset. Even in neurologically intact patients, radiological confirmation of tethering may justify early surgical intervention to prevent future neurological deterioration. Careful radiological assessment and multidisciplinary coordination are essential to optimize long-term functional outcomes.
Pediatric neurocritical care (PNC) has emerged as a specialized field aimed at improving outcomes for children with neurological conditions. Despite advances, pediatric neuro-oncology remains underrepresented within PNC literature. To describe the clinical and severity profiles of children admitted over a decade to a Pediatric Neurocritical Care Unit (PNCU) at the Instituto do Cérebro Paulo Niemeyer (IECPN), with special focus on patients with central nervous system (CNS) tumors. This retrospective, descriptive observational study included patients admitted to the PNCU from September 2013 to August 2023. Demographic, clinical, and severity data were collected, ICU and hospital length of stay, mortality, and discharge disposition. Comparative analyses were performed between patients with and without CNS tumors. A total of 1079 admissions were analyzed, with a median age of 6 years. Most admissions were surgical, predominantly for tumor resection (34
Background Occult spinal dysraphism (OSD) represents a group of congenital spinal anomalies in which neural tissue remains covered by intact skin. Because the skin and nervous system share a common ectodermal origin, midline lumbosacral cutaneous lesions are frequently associated with underlying spinal abnormalities. Identifying which cutaneous markers warrant spinal imaging remains clinically important, particularly in children presenting with bowel or bladder dysfunction. Objective To evaluate and emphasize the importance of the possible association between various midline lumbosacral cutaneous lesions and occult spinal dysraphism, and to determine when spinal imaging should be recommended in pediatric patients. Methods This retrospective case series included pediatric patients presenting between January 2019 and December 2024 with congenital midline lumbosacral cutaneous lesions and/or deviation of the gluteal furrow, and urinary or bowel symptoms. Patients with open neural tube defects were excluded. Clinical data were obtained from medical records, and imaging findings were reviewed by a team of pediatric radiologists and neurosurgeons. Spinal magnetic resonance imaging (MRI) and/or spinal ultrasound (USG) were performed to detect occult spinal anomalies. Descriptive statistical analysis was used to summarize clinical characteristics and imaging outcomes. Results Twenty-six patients were included (65.4% females, mean age 2.3 years). Isolated cutaneous lesions were present in 65.4% of patients, while 34.6% had multiple lesions. The most common cutaneous marker was deviation of the gluteal furrow (38.5%), followed by port-wine stain and dermal sinus (each 19.2%). MRI was performed in 80.8% of patients and ultrasound in 30.8%. Occult spinal dysraphism was confirmed in 10 children (38.5%). Tethered cord and spinal lipomas were the most frequent abnormalities, each identified in 70% of OSD cases. OSD was more frequently associated with multiple cutaneous markers compared with isolated lesions. Conclusion Midline lumbosacral cutaneous lesions, particularly when multiple or associated with high-risk markers such as lipomas or dermal sinuses, have a significant association with occult spinal dysraphism.
