Primary brain tumors are the most common solid neoplasms in children and young adults. Surgical resection remains the cornerstone of treatment, with extent of resection and histopathology serving as main prognostic factors. While magnetic resonance imaging (MRI) is the gold standard for neuroimaging and surgical planning, [18F]-fluoroethyl-L-tyrosine positron emission tomography ([1⁸F]-FET PET) may provide complementary metabolic insights. We evaluated the role of [18F]-FET PET combined with preoperative MRI in the management of pediatric brain tumors. We retrospectively selected from a single-institutional registry 12 patients aged 16 years or younger who underwent surgical treatment for brain tumors at a single tertiary pediatric center between 2013 and 2024. All patients underwent preoperative [^18F]-FET PET in addition to MRI. PET findings were used to support biopsy targeting, surgical resection planning, and follow-up assessment. Histopathological and molecular diagnoses were reviewed according to the 2021 World Health Organization (WHO) classification of central nervous system. Histopathological diagnoses included low-grade gliomas and glioneuronal tumors, high-grade gliomas, diffuse midline glioma, and embryonal tumors. [^18F]-FET PET identified metabolically active neoplastic tissue in 11 of 12 patients. One false-positive finding occurred in a previously treated lesion and was ultimately attributed to reactive gliosis. PET/MRI integration supported surgical decision-making in all cases and proved particularly useful in differentiating tumor recurrence or progression from treatment-related changes during follow-up. In this single-institution pediatric series, [1⁸F]-FET PET had high utility in preoperative assessment of pediatric brain tumors. Its integration with MRI provided valuable additional data to guide surgical targeting, especially when conventional imaging was inconclusive. These findings support the use of [1⁸F]-FET PET in precision pediatric neuro-oncology, but considering the limited number of patients enrolled in this study, larger prospective studies are needed to define its role in pediatric neuro-oncology.

There are no specific guidelines for posterior cranial fossa decompression (PCFD) in asymptomatic Chiari Malformation Type I (CM-I) patients with autism spectrum disorder (ASD). However, some studies suggest that surgery for symptomatic CM-I may improve ASD symptoms. This study aims to identify skull and brain morphometric parameters that could predict surgical outcomes in symptomatic CM-I with ASD, using artificial intelligence (AI). This study included pediatric patients diagnosed with both symptomatic CM-I and ASD who underwent posterior cranial fossa (PCF) surgery. Eleven morphometric parameters were measured using computed tomography (CT) and magnetic resonance imaging (MRI) scans, including cerebellar tonsil descent, tentorium length and angle, cerebellum-to-PCF area ratio, PCF-to-cerebrum area ratio, PCF height and diameter, and various distances involving the corpus callosum, pons, fastigium, foramen magnum, and clivus length. ASD symptom changes were assessed through phone interviews and outpatient evaluations. A binary tree classifier AI model was used to identify patients who improved post-surgically. Our analysis showed that patients with a larger tentorium angle experienced some improvements in ASD symptoms after surgery, whereas those with a significantly smaller tentorium angle showed no improvement. AI identified a tentorium angle of 89.55° as a potential cut-off for distinguishing between outcome groups. No other morphometric parameters significantly influenced ASD symptom outcomes. This study evaluates the relevance of the tentorium angle width as a potentially valuable MRI-based morphometric parameter that could guide neurosurgeons in the decision-making process for this unique patient population. These findings may contribute to a more tailored approach for managing patients with CM-I and coexistent ASD.

Spina bifida is a common congenital anomaly caused by the incomplete fusion of the caudal neural tube, classified as spina bifida aperta (open) with a visible lesion and spina bifida occulta (closed) without external lesions. Its causes are diverse, including genetic factors, reduced intake of folate and nutrients, maternal obesity, diabetes and certain teratogens. Most cases may be prevented with maternal folic acid supplementation. Closed spinal dysraphism is harder to diagnose due to the lack of evident lesions, but may be suspected in asymptomatic newborns with midline skin signs. The paper describes the case of a newborn presenting with an atypical lumbosacral dimple linked to a lumbosacral vascular malformation. Radiological imaging (US and MRI) facilitated early detection and treatment of the neural tube defect, preventing progressive neurological and orthopaedic damage linked to tethered cord syndrome.

Deep Brain Stimulation (DBS) is a therapeutic strategy for the treatment of dystonia, also in pediatric settings. It acts selectively on deep areas such as the internal Globus Pallidus (GPi). In this study, we report a multimodal analysis carried out on a young patient suffering from generalized dystonia who underwent bilateral DBS implantation at the level of the GPi. The objective was to identify the neurophysiological correlates of dystonia. Through the synchronized recording of deep cerebral (Local Field Potentials, LFP), electroencephalographic, (EEG), and electromyographic (EMG) signals, dystonic events were identified and analyzed in different stimulation configurations (including ring mode and directional settings using contacts either proximal to or distal from GPi) and at various levels of stimulation intensity. Spectral analysis was performed on the signals to identify frequency bands associated with dystonic events and to investigate the effects of stimulation. The findings suggest that a personalized approach to DBS may improve the therapeutic management of pediatric dystonia.

Deep brain stimulation (DBS) of nucleus ventralis intermedius thalami (Vim) is a validated technique for the treatment of essential tremor (ET) in adults. Conversely, its use for post traumatic tremor (PTT) and in paediatric patients is still debated. We evaluated the efficacy of Vim-DBS for lesional tremor in three paediatric patients with drug-resistant post-traumatic unilateral tremor. We retrospectively collected data regarding three patients with unilateral tremor due to severe head injury, with no MRI evidence of basal ganglia lesions. The three patients underwent stereotactic frame-based robot-assisted DBS of Vim contralateral to the tremor side. Mean follow-up was 48 months (range: 36–60 months). Tremor was reduced in all patients with a better control of voluntary movements and improvement of functional status (mean FIM scale improvement + 7 points). No surgical complications occurred. Unilateral contralateral DBS of Vim could be efficacious in post-traumatic tremor, even in paediatric patients and should be offered in PTT drug-resistant patients.

INTRODUCTION:Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm that affects patients, predominantly males aged 40-70 years, with very heterogeneous clinical presentation and prognosis. In 2020, Goyal et al. proposed consensus recommendations for the management of patients with ECD, remarking on the exceptional presentation of the disease in the pediatric population. CASE PRESENTATION:The first patient, a 20-year-old male, underwent cervical laminectomy and partial removal of a cervical spine lesion, initially apparently consistent with cervical schwannomas. The second patient, a 9-year-old female, received surgery for an extra-axial lesion of the greater sphenoid wing, radiologically consistent with a meningioma. CONCLUSION:At present, 15 pediatric cases have been reported in the literature with involvement of the central nervous system, with no consensus on the diagnostic and therapeutic management, as Pegoraro et al. evidenced in their pediatric multicenter case series. The present article adds two new cases of ECD with onset in childhood and young adulthood, who received the diagnosis after neurosurgical procedures.

We provide an extensive review of clinical features, diagnosis, and treatment of primitive facial nerve tumors in children, and report 2 recent personal observations.We conducted a comprehensive literature search through PubMed, Medline, and ScienceDirect and collected information on patients' age, symptoms, tumor types and sites, diagnostic procedures, surgical approaches, and outcomes.Overall, we reviewed 26 pediatric cases from 20 papers.About 69.2% of children presented with some degree of facial palsy.Other symptoms included hearing loss, dizziness, and tinnitus.84.6% of tumors were schwannomas, followed by meningiomas, epithelioid hemangioendothelioma, and germ cell tumors.The geniculate ganglion was the most commonly affected segment of the facial nerve.A total of 92.3% of children received surgery as complete or partial tumor resection.Facial nerve function improved in 26.9% of children.No tumor recurrence was reported.Facial nerve tumors are extremely rare in children but should be considered in the differential diagnosis of facial palsy, even in newborns.Audiometric and radiologic examinations are necessary; radiologic imaging allows to determine tumor localization, and the correct surgical approach surgery is suggested in almost all cases.

We report the case of a 16-year-old girl presenting with spinal clear-cell multiple meningiomas (CCMs). In view of this presentation, we sequenced a bioinformatic panel of genes associated with susceptibility to meningioma, identifying a germline heterozygous variant in SMARCE1. Somatic DNA investigations in the CCM demonstrated the deletion of the wild-type allele (loss of heterozygosity, LOH), supporting the causative role of this variant. Family segregation study detected the SMARCE1 variant in the asymptomatic father and in the asymptomatic sister who, nevertheless, presents 2 spinal lesions. Germline heterozygous loss-of-function (LoF) variants in SMARCE1, encoding a protein of the chromatin-remodeling complex SWI/SNF, have been described in few fa-milial cases of susceptibility to meningioma, in particular the CCM subtype. Our case confirms the role of NGS in investigating predisposing genes for meningiomas (multiple or recurrent), with specific regard to SMARCE1 in case of pediatric CCM. In addition to the age of onset, the presence of familial clustering or the coexistence of multiple synchronous meningiomas also supports the role of a genetic predisposition that deserves a molecular assessment. Additionally, given the incomplete penetrance, it is of great importance to follow a specific screening or follow-up program for symptomatic and asymptomatic carriers of pathogenic variants in SMARCE1.

During the last decade, there has been renewed interest in stereotactic approaches to diffuse intrinsic pontine gliomas (DIPGs) in children, due to the development of new concepts in molecular biology and management, and subsequent need for tissue sampling. Stereotactic frame-based and robot-assisted techniques are associated with reduced target error and have been incorporated into standard practice at our institution. Four children (age 2–7 years) underwent a robot-assisted frame-based transcerebellar approach using the Leksell G frame coupled with Renishaw’s neuromate® stereotactic robot. The procedures included 3 biopsies (two brainstem tumors and one cerebellar hemispheric lesion) and 1 depth electrode implantation into a low-grade tumor remnant (ganglioglioma) of the middle cerebellar peduncle causing drug-resistant epilepsy in a young girl. Targeting was based on MRI, and in one case, 18F-FET-PET was coregistered to MRI to improve sampling accuracy. The frame was applied 180° rotated compared to standard orientation, and patients were positioned prone during surgery and stereotactic preoperative CT scan. Postoperative CT scan ruled out complications and was coregistered to preoperative MRI to check the target accuracy. No complications occurred, and targeting was accurate in all cases. All tissue samplings provided proper histology; depth electrode EEG exploration was diagnostic and led subsequent resective surgery. According to our experience, the transcerebellar frame-based robotic stereotactic approach to the cerebellum and the brainstem is feasible, safe, and effective even in young children.

Background: Acute traumatic epidural hematoma (EDH) is a complication in 2–3% of pediatric head injuries. Surgery is mandatory in symptomatic cases; otherwise, conservative treatment is a valid approach, especially in the pediatric population. Ossified epidural hematomas (OEHs) have been reported in the pediatric population as a rare complication of conservative EDH management, although the exact incidence remains unknown. The progressive increase in conservative management may lead to increases in the OEH incidence over the next few years. Our study aimed to systematically review OEH incidence, management strategies, characteristics (thickness, inner/outer calcifications), complication rates, time to surgery after the EDH diagnosis, and clinical outcomes. Summary: A systematic review was conducted in accordance with the PRISMA guidelines. Studies reporting diagnoses and clear descriptions of OEH after EDH in pediatric patients were considered eligible. Sixteen studies, including 18 pediatric patients aged 0–18 years, were included. Head trauma was the most common cause of OEH. Seven (38.8%) OEHs were treated less than 1 month after EDH diagnosis. Surgery was performed in 17 cases (94.44%), while 1 asymptomatic case (5.56%) was managed conservatively. Key Messages: Surgery was the most commonly used treatment for OEH. Data for conservative treatment of OEH are limited. Magnetic resonance imaging or ultrasound within the first 2 months, to check for EDH resolution, may be crucial to rule out complications in pediatric patients.

Background: Myelomeningocele (MMC) is the most common open spinal dysraphism (OSD) and is typically associated with other clinical and/or radiological abnormalities. Chiari type 2 malformation (CM-II) is observed in 80% to 96.7% of patients, and approximately 80% of cases involve posterior fossa abnormalities leading to hydrocephalus. MMC patients can present with syringomyelia, which usually improves by treating hydrocephalus. However, a minority of patients develop a progressive form of syringomyelia requiring more invasive treatments. Through this retrospective observational study, our objective is to elucidate the incidence of syringomyelia, its symptomatic manifestations, and the associated radiological and clinical characteristics in pediatric patients diagnosed with myelomeningocele Methods: A retrospective analysis was conducted on the medical records of 39 pediatric patients diagnosed with myelomeningocele at a tertiary referral institute. The study encompassed individuals born between 1997 and 2022. Descriptive statistics were employed to explore epidemiological aspects and clinical features. Additionally, incidence rates of syringomyelia and its progressive variants were calculated, with a focus on their correlation with diagnoses of hydrocephalus, Chiari type 2 malformation (CM-II), and spinal cord tethering. Results: Over the last twenty-five years, the incidence of syringomyelia in pediatric patients affected by MMC was 46.2%. The incidence of its progressive forms was 16.7%. Two patients improved after the management of hydrocephalus with secondary Endoscopic Third Ventriculostomy (ETV), and only one patient required syrinx-shunting. Conclusions: The occurrence of syringomyelia among patients with myelomeningocele (MMC) in our center aligns with reported rates in existing literature. Our findings indicate that active hydrocephalus, leading to a deterioration of hindbrain herniation, appears to be a significant factor in the development of progressive symptomatic syringomyelia within this population.

Introduction: Although still considered quite harmless, nonpowder guns (NPG) may cause severe head injuries in children. We present the case of a depressed skull fracture with superior sagittal sinus involvement following NPG injury. Decision-making and surgical management are discussed, and the current literature concerning NPG-related pediatric head injuries is reviewed. Case Presentation: A 4-year-old boy hit by a compressed-air rifle came to our center. CT scan showed a depressed skull fracture of the occipital bone on the midline and intracranial penetration of some fragments of the pellet. CT angiography documented a compression of the superior sagittal sinus without thrombosis. Soon after hospital admission, the patient showed deterioration of the neurological status suggesting intracranial hypertension. Surgery was performed with fracture elevation, removal of metal fragments, and wound debridement. The patient had a full recovery without subsequent neurological deficits. Discussion/Conclusion: Modern airguns may produce severe penetrating head injuries in children. Parents and physicians should be aware of the danger of NPG. Depressed skull fracture and dural venous sinus involvement can occur, and even a stable neurological condition may worsen. In such instances, a thorough preoperative assessment including vascular imaging is mandatory. The surgical risk is not negligible due to the possible injury of the sinus wall and subsequent intraoperative bleeding. This has to be weighted against potential complications of the penetrating injury, such as infection, sinus thrombosis, and intracranial hypertension. Our case suggests that prompt surgical treatment can be a safe and effective option.

The COVID-19 outbreak has dramatically changed the organization of Pediatric Neurosurgery all over the world. The departments involved developed similar plans to maintain emergency surgeries without reducing clinical activities. The Association of Pediatric Neurosurgeons wrote different memoranda to detail the surgical procedures not to be postponed with special attention given to high-risk pathology for COVID-19 contamination, like trans-naso-sphenoidal surgery. On this basis, we have conducted a complete literature review focusing on many topics: hospital organization, patients and parents screening, surgical indication criteria, outpatient clinic and teleconsultation, telematic conference and meeting, fellowship and training, and virtual multidisciplinary meeting.

Introduction Loculated hydrocephalus is a complex condition in which different non-communicating compartments form within the ventricular system due to different etiology, mainly intraventricular hemorrhage and infection. Since the end of the twentieth century, neuroendoscopy has been explored as a therapeutic option for loculated hydrocephalus with non-univocal results. Methods We performed a retrospective analysis of 90 patients who underwent endoscopic treatment for loculated hydrocephalus from January 1997 to January 2021 (mean age: 2 years, range 7–21). We included 37 (41.1%) children with multiloculated hydrocephalus, 37 (41.1%) with isolated lateral ventricle, 13 (14.4%) with excluded temporal horn, and 3 (3.3%) with isolated fourth ventricle. We compared our results with those available in literature. Results A mean of 1.91 endoscopic procedure/patient were performed (only one endoscopy in 42.2% of cases). Complications of neuroendoscopy and of shunt surgeries were recorded in 17 (18.9%) and 52 (57.8%) children, respectively. Twenty-six (28.9%) children were shunt-free at the last follow-up, 47.8% have only one shunt. Discussion The first goal of neuroendoscopy is to increase the rate of shunt-free patients but, when it is not possible, it aims at simplifying shunt system and reducing the number of surgical procedures. In our series, neuroendoscopy was able to achieve both these goals with an acceptable complication rate. Thus, our results confirmed neuroendoscopy as a valid tool in the long-term management of loculated hydrocephalus. Neuronavigation and intraoperative ultrasound could increase the success rate in cases with distorted anatomy.

Although still considered quite harmless, nonpowder guns (NPG) may cause severe head injuries in children. We present the case of a depressed skull fracture with superior sagittal sinus involvement following NPG injury. Decision-making and surgical management are discussed, and the current literature concerning NPG-related pediatric head injuries is reviewed.A 4-year-old boy hit by a compressed-air rifle came to our center. CT scan showed a depressed skull fracture of the occipital bone on the midline and intracranial penetration of some fragments of the pellet. CT angiography documented a compression of the superior sagittal sinus without thrombosis. Soon after hospital admission, the patient showed deterioration of the neurological status suggesting intracranial hypertension. Surgery was performed with fracture elevation, removal of metal fragments, and wound debridement. The patient had a full recovery without subsequent neurological deficits.Modern airguns may produce severe penetrating head injuries in children. Parents and physicians should be aware of the danger of NPG. Depressed skull fracture and dural venous sinus involvement can occur, and even a stable neurological condition may worsen. In such instances, a thorough preoperative assessment including vascular imaging is mandatory. The surgical risk is not negligible due to the possible injury of the sinus wall and subsequent intraoperative bleeding. This has to be weighted against potential complications of the penetrating injury, such as infection, sinus thrombosis, and intracranial hypertension. Our case suggests that prompt surgical treatment can be a safe and effective option.

Introduction: Achondroplasia is a genetic disorder characterized by defects in the development of endochondral bone resulting in skeletal abnormalities like stenosis of the foramen magnum and of the spine, shortened limb bones, and macrocephaly. Congenital spinal stenosis is frequent and due to premature fusion of the pedicles to the laminae. Case Presentation: We report a case of neurogenic bladder and fecal incontinence due to lumbar stenosis successfully treated with L1-L5 partial laminectomy and foraminotomy in a 7-year-old achondroplasic child. Discussion/Conclusion: To our knowledge, this is the first case report of exclusive neurogenic bladder and fecal incontinence in an achondroplasic child. Neurogenic bladder and fecal incontinence without motor impairment may be early and exclusive clinical findings of lumbar stenosis in children with achondroplasia.

BACKGROUND:Vagus nerve stimulation (VNS) represents a valid therapeutic option for patients with medically intractable seizures who are not candidates for epilepsy surgery. Even when complete section of the nerve occurs, stimulation applied cranially to the involved nerve segment does not preclude the efficacy of VNS. Complete vagus nerve section with neuroma causing definitive left vocal cord palsy has never been previously reported in the literature.OBSERVATIONS:Eight years after VNS implant, the patient experienced worsening of seizures; the interrogation of the generator revealed high impedance requiring surgical revision. On surgical exploration, complete left vagus nerve section and a neuroma were found. Vocal cord atrophy was found at immediate postoperative laryngeal inspection as a confirmation of a longstanding lesion. Both of these events might have been caused by direct nerve injury during VNS surgery, and they presented in a delayed fashion.LESSONS:VNS surgery may be complicated by direct damage to the left vagus nerve, resulting in permanent neurological deficits. A complete section of the nerve also enables an efficacious stimulation if applied cranially to the involved segment. Laryngeal examination should be routinely performed before each VNS surgery to rule out preexisting vocal cord dysfunction.

Ventriculoatrial (VA) and ventriculopleural (VPL) shunts are used as alternatives when CSF diversion to the peritoneal compartment with a ventriculoperitoneal (VP) shunt is not possible. The objective of this study is to compare directly the shunt survival and complications for both procedures in this setting in children. A retrospective analysis of 54 consecutive patients who underwent VA (36) or VPL (18) shunt insertion between January 2002 and December 2017 was conducted. The overall mean follow-up was 4.1 (SD 4.3) years, 2.8 (SD 4.1) for VPL and 4.7 (SD 4.4) for VA shunts, respectively (p = 0.11). Twenty-four (66.7%) patients in the VA group and 9 (50.0%) in the VPL group underwent shunt revision (p = 0.236); mean number of revisions was 2.2 (SD 3.0) and 0.94 (SD 1.4) in the VA and VPL groups (p = 0.079). Median time to failure was 8.5 (IQr 78, range 0–176) months for VA and 5.50 (IQr 36, range 0–60) for VPL shunts (log rank (Mantel-Cox) 0.832). Shunt survival at 3, 6, 12 and 30 months was 60.6, 51.5, 36.4 and 27.3%, respectively, for VA and 56.3, 43.8, 37.5 and 37.5% for VPL shunts (log rank (Mantel-Cox) test value 0.727). The infection rate was 13.8% for VA and 5.6% for VPL shunts (p = 0.358). Four patients with VPL shunts (22.2%) developed pleural effusions. Fourteen deaths (25.9%) were recorded during follow-up, 8 (22.2%) in the VA and 6 (33.3%) in the VPL group (p = 0.380); two of the deaths in the VA group were shunt-related. This study demonstrates that the outcomes of VA and VPL shunts, when used as second-line surgical treatment in paediatric hydrocephalus, were similar, as were the revision, infection and survival rates. The shorter longevity of these shunts compared with the general shunted population may reflect the complex nature of these children.