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Thalamic tumors are deep-seated lesions. Recent improvements in therapeutic approaches and surgical techniques have allowed a more accurate approach to these lesions and a reduction in morbidity and mortality. In this article, the various surgical approaches for the resection of thalamic tumors are described. Each of these approaches has its own indications and risk of complications. Resection of thalamic tumors needs specific anatomical knowledge, especially the vascular anatomy of the region and the thalamic peduncles.

A new entity of gliomas, named "diffuse midline glioma (DMG), H3K27M mutant (grade IV)," which represents a specific molecular profile, was introduced to the World Health Organization (WHO) classification 2016 of central nervous system tumors. Many midline localizations have been described for this glioma, and mainly the hypothalamus, pons, thalamus, and spinal cord are sites of predilection in pediatric and young adult patients. We report the case of spinal intramedullary DMG, H3K27M mutant (WHO grade IV), that showed an unusual presentation with multiple vertebral metastases.

Purpose: this study investigated the risk for children with non-syndromic craniosynostosis to develop behavioral problems during school age determined by the type of craniosynostisis, age at first surgery, and number of surgeries. Method: final sample consisted of 43 children aged between 6 years and 8 months and 17 years and 1 month (M = 10 years and 5 months). Behavioral problems were assessed with Child Behavioral Checklist (CBCL). Results: our sample had higher scores on the CBCL than the general population; specific elevations were observed including somatic complaints, aggressive behavior, social problems, attention problems, and thought problems and rule-breaking behavior. Behavioral functioning varied by number of surgical procedures, type of craniosynostosis, and age at first surgery. Conclusion: for school-aged NSC children's behavioral functioning, diagnosis specific patterns especially impacted by the first age of the surgery and number of surgeries., NA

Objective Diffuse Midline Gliomas (DMG), H3 K27M-mutant have the poorest prognosis among all pediatric high-grade gliomas, with a median survival of 9-11 months. Although radiotherapy (RT) is standard treatment for these tumors, unfortunately there has been no approved and effective treatment which completely diminishes the tumor yet. In our clinic, we started an up-to-date approach to manage DMG, which is adjuvant fractionated external beam radiotherapy along with ONC 201 after tissue diagnosis. Methodology Between January 2016 and June 2021, a total of 11 patients with H3 K27M-mutant diffuse midline glioma, diagnosis confirmed by Next-Generation Sequencing (NGS) were enrolled in study. All patients received ONC201 orally once a week following radiotherapy. Safety, and radiological evaluations were regularly assessed every 12 weeks. Results Among the 11 patients, the median age of diagnosis was 5. Seven (63.6%) patients were male and 4 (36.4%) were female. Primary lesions were localized in the pons in 5 (45.5%) patients, unilateral thalamus (2 on the left, 1 on the right) in 3 (27.3%) patients, bilateral thalamus in 2 (18.2%) patients, and temporo-insular in 1 patient (9.1%). Median progression-free interval was 10 months and median overall survival was 16 months. Conclusion Diffuse midline glioma has dismal prognosis. None of the treatment options made any dramatic changes in disease course during last 30 years. In our series, diffuse midline glioma patients who had ONC201 tend to have few months more progression free and overall survival (16 vs 11 months) in comparison to patients who had classical treatment in literature. As a neurooncology team, we strongly advocate to obtain tissue samples from diffuse tumors, to establish definite diagnosis and to perform NGS

Objective Pediatric high grade gliomas(HGG) have dismal prognosis with median survival of 9-15 months after standard radio-chemptherapy. Recent molecular investigations revealed a missmatch repair defect called Constitutional Mismatch Repair Deficiency (CMMRD), which induce pediatric HGG. In CMMRD, there are mutations at least one of the mismatch repair(MMR) genes in both tumoral and non-tumoral DNA. Patients generally have cafe au lait spots resembling the ones in NF-1. Methodology Forty-four pediatric high-grade glioma cases operated in our clinic between 2015-2021 were included in the study. PMS2, MLH1, MSH6, MSH2 immunohistochemical antibodies were applied to the sections prepared from paraffin blocks with tumors of these 44 cases. Next generation Sequencing (NGS) Custom Panel for Brain Tumors was performed with DNA and RNA obtained from neoplastic tissue of 2 cases and germline NGS analysis was performed with DNA obtained from peripheral blood in 1 case. Results MMR protein expression loss was detected in 11 (25%) cases. In 5 (45%) of these 11 cases, MMR protein loss was detected in both neoplastic and non-neoplastic tissue, and these cases were considered as CMMRD. NGS performed in 2 of these 5 cases revealed a hypermutant profile. At least one MMR protein loss was found only in the neoplastic tissue in 6 (55%) of 11 cases, and PMS2 deficiency was the most common. In 1 of these 6 cases, MSH6 deficiency was shown as germline by NGS. Conclusion CMMRD and MMRD, are disorders with close relationship with pediatric high grade gliomas. Since CMMRD cases also may have cafe au lait spots, they should not be misdiagnosed as NF 1. Temozolomide induce more aggressive tumors in CMMRD ve MMRD, therefore its use is not suggested in those cases. Preliminary literature data advocate use of immunotherapy instead. All pediatric HGG cases should be evaluated for CMMRD and MMRD with molecular investigations to understand their biology.

Objective: This study aims to investigate the neurourological and urodynamic findings of children with cerebral palsy (CP) that referred for dorsal rhizotomy surgery (DRS). Materials and Methods: All children with CP who were scheduled for selective DRS were assessed with a detailed medical history, physical exam, urinalysis and urodynamic studies to assess bladder function and urinary problems. Urodynamic studies included filling and voiding cystometry, detrusor leak point pressure, external anal sphincter electromyography, flow rate and residual urine volume. All investigations and definitions relied on the standardisation of the International Continence Society. Results: Overall, 24 boys and 10 girls were evaluated. The mean age of boys, and girls and the study group was 6.6 (1.7-9.8), 6.5 (3.5-11.4) and 6.6 (1.7-11.4) years, respectively. The most common complaints of the study group were urinary incontinence (58.8\%), encopresis (32.4\%) and constipation (17.6\%) and 41.2\% of patients needed diapers due to these problems. Twenty-five per cent (n=5) of male patients had an undescended testis. The most common clinical conditions at urodynamics, were low bladder compliance (85.3\%), detrusor overactivity (67.6\%), hyposensitive bladder (52.6\%) and low bladder capacity (41.2\%). Conclusion: Upper motor neuron lesions, like CP, may present with various urodynamic findings. However, patients with CP are not routinely seen by urologists. Our findings revealed serious neurological problems in children referred for DRS. Therefore, every child with CP who has a DRS plan should undergo a detailed urological examination at least once before the procedure.

Objectives The prevalence of low-back pain (LBP) in adolescents ranges from 7 to 72%. We aimed to define the radiologic characteristics of the lumbar spine in children and adolescents with LBP with/without leg pain. Methods Two hundred and fourteen children and adolescents, who were born between 2001 and 2009 and had lumbar spine MRI for LBP with/without leg pain, were evaluated in terms of intervertebral disc degeneration (IVDD), end-plates and paraspinal muscle changes on lumbar spine MRIs. Results Severe IVDD was detected at all lumbar levels except for L2-L3. Modic changes were present in 4.2% of the patients. Modic changes were more common in patients with severe IVDD than in those with mild-to-moderate IVDD. Severe IVDD was significantly associated with Modic changes at the corresponding L1-L2 and L3-L4 disc levels. Girls had significantly more fatty infiltration in the paraspinal muscles when compared to boys. The risk of having severe IVDD concomitant with Modic changes was high [odds ratio (OR), 8.6]. The OR was 20.7 for predicting the presence of severe IVDD at any level if Modic changes presented particularly at the L3-L4 level. The ORs of Modic changes presented at any lumbar level at the background of fat-infiltrated multifidus at L3-L4 and L4-L5 levels were 8.3 and 9.1, respectively. Conclusions Fatty infiltration in the paraspinal muscles and IVDD were closely associated with Modic changes in children and adolescents with LBP. Lumbar IVDD in children and adolescents could be the result of a mechanical pathology.

Objective: Apert syndrome is a rare and understudied craniofacial condition with regard to its psychosocial impact on children and their parents. Due to the lack of studies focusing solely on the social experiences of children and families with Apert syndrome, it is difficult to develop interventions and strategies to support well-being and positive adjustment for this particular population. This study addressed this gap by assessing the unique social experiences of parents who are raising their children with Apert syndrome including difficulties they face and strategies they use to cope with challenges. Design: Descriptive qualitative study using thematic analysis. Participants: Participants included 21 parents of 12 children (aged 1-12 years) with Apert syndrome (9 couples, 2 fathers, and 1 mother) who were recruited from a pediatric neurosurgery unit in Turkey. Results: The qualitative analysis yielded four main themes describing the experiences of parents including (1) social challenges; (2) coping with negative reactions; (3) promoting socialization and independence; and (4) sources of strength. Conclusions: Parents reported several challenges, including social stigmatization, and utilized a range of strategies to support both their own and their child’s positive adaptation and resiliency, including the use of religion and reliance on their spouses for support. Results offer clinically relevant insights about the strengths and challenges of families coping with Apert syndrome.

Recent advances in the genomic study have revolutionized the discovery of molecular biomarkers in glioma not only to aid in targeted therapy but also to prognosticate character of tumor and outcome of a patient.The usually benign nature of pilocytic astrocytoma (PA) can now be challenged with the discovery of genomic alterations that could promote more aggressive clinical behavior. CDKN2A deletion and ATRX gene inactivation or mutation have been the most common molecular alterations in worse prognosis.We will discuss a case of pilocytic astrocytoma showing the progressive recurrence with pilomyxoid features and the presence of TERT promoter mutation.

Advances in preoperative brain magnetic resonance imaging (MRI) have increased our ability to predict tumor pathology (even grade) and to make a proper surgical plan accordingly. Perioperative brain MRI has been shown to increase tumor resection rates in glial and pituitary tumors. Besides, perioperative MRI enables us to reach deep-seated tumor location in a safe trajectory. In this chapter, we present our clinical experience in preoperative and perioperative brain MRI modalities by presenting three cases.

Tumors originating from the midbrain, pons and medulla are collectively named as brainstem tumors. Their prevalence in the pediatric age group is around 10–20%. They may grow as focal or diffuse lesions. Unfortunately, the majority of these lesions have bad prognosis. In general sense, acute onset of symptoms with multiple cranial nerve palsies indicates worse prognosis. The most frequent symptom of a brainstem tumor is pyramidal weakness. Other symptoms are nausea, vomiting, balance problems and cranial nerve palsies. Patients with cranial nerve palsies may have double vision, conjugate gaze palsy, swallowing difficulties, dropped shoulder, and problems with tongue movements. Papilledema is not a usual sign of brainstem tumors because it is generally a late problem in these patients. Less frequently, these patients may have nystagmus and other rare brainstem syndromes involving the pons and the midbrain.

Here we are presenting a unique case of malignant triton tumor of the trigeminal nerve in a 4-year-old boy who presented with diplopia and ptosis. Near total excision of the tumor was performed, and adjuvant chemotherapy and radiotherapy were administered. The patient is in good health and has no evidence of clinical and radiological tumor recurrence for 22 months.

BOZKURT, SUHEYLA UYAR uyar/0000-0002-5947-947X WOS: 000419966300020 PubMed: 29063957 Pediatric high-grade gliomas (pHGGs) constitute almost 15% of all childhood brain tumors. Recurrent mutations such as H3K27M mutation in H3F3A and HIST1H3B genes encoding histone H3 and its variants were identified in approximately 30% of pediatric glioblastomas. This study aimed to ascertain the morphological and molecular characteristics of pHGGs with H3K27M mutation. In total, 61 cases of pHGGs (anaplastic astrocytoma, 12; glioblastomas, 49) from four university hospitals were studied. The histomorphological features were examined and immunohistochemistry was performed to evaluate the mutation status of H3K27M, ATRX, IDH1, BRAF V600E, and p53 genes. The study comprised 25 females and 36 males (age range, 1-18 years) with a clinical follow-up of up to 108 months. From the total, 31 patients were positive for H3K27M mutation located in the midline, mostly in the pons and thalamus. H3K27M mutation was commonly associated with ATRX loss (32.3%) and p53 (74.2%) immunoreactivity with a co-expression rate of 25.8%. While IDH1 mutation was not detected in pHGGs with H3K27M mutation, BRAFV600E mutation was rarely observed. Among the various histomorphological features, increased number of mitosis, increased Ki-67 proliferation index, and palisading and geographical necrosis along with small cell patterns were significantly associated with the H3K27M wild-type tumors. Focal infarct-like necrosis and pilomyxoid morphology was significantly associated with these tumors. H3K27M mutation occurs exclusively in pHGGs arising from the midline and presents with varied histomorphological features ranging from low-grade pilomyxoid astrocytoma to highly pleomorphic glioblastoma along with ATRX loss and p53 mutations.

Hypothalamic hamartomas (HH) generally present with gelastic seizures. It is very unusual for a pediatric patient with HH to present with infantile spasms (IS). Here we present a 6-month-old boy diagnosed with IS whose brain magnetic resonance imaging (MRI) showed an 18 × 18 × 16 mm mass in the hypothalamus. His seizures did not respond to antiepileptic treatment with vigabatrin and valproic acid. He had disconnective surgery for HH. Immediately postoperatively, his seizures subsided and he has now been seizure-free for 2 years. Although hypothalamic hamartomas generally present with gelastic seizures, they should also be considered in the differential diagnosis of infantile spasms.

Posterior fossa ependymomas (PFE) are among the most frequently occurring solid tumors in children. Their definitive treatment is surgical excision and adjuvant radio-chemotherapy. This study aimed to investigate prognostic effects of age, H3K27me3 status, extent of resection, radiation treatment (RT), Ki67 index, WHO grade, and ATRX and H3K27M mutations in PFE patients. This retrospective study included 42 pediatric patients with PFE who had undergone operation at our institution between 1996 and 2018. Patient demographics and treatment information were obtained from patient notes. Information on radiological location of tumors (median vs paramedian), extent of tumor resection, and recurrence was obtained from preoperative and postoperative magnetic resonance imaging. Formalin-fixed paraffin-embedded tumor samples were evaluated for H3K27me3 immunostaining, Ki67 index, WHO grades, and ATRX and H3K27M mutations. Tumor samples with global reduction in H3K27me3 were grouped as posterior fossa ependymoma group A (PFA) and those with H3K27me3 nuclear immunopositivity as posterior fossa ependymoma group B (PFB). We evaluated the cohort’s 5-year progression-free survival (PFS) and overall survival (OS). There were 20 (47.6%) female and 22 (52.4%) male patients in the cohort. The mean age of patients was 4.4 (range, 0.71–14.51) years. Overall, tumors in 31 (73.8%) and 11 (26.2%) patients were found to be PFA and PFB, respectively. There was no statistically significant age or sex difference between PFA and PFB. All patients received chemotherapy, whereas only 28 (66.6%) received RT. The WHO grades of PFA were statistically higher than those of PFB. There was no significant difference between PFA and PFB in terms of extent of resection, disease recurrence, and survival parameters. Nine of 42 tumor samples had ATRX mutations. One patient with PFA showed H3K27M mutation. Age, WHO grade, H3K27me3 status, and RT had no effect on patients’ PFS and OS. Patients with total surgical excisions had significantly better PFS and OS rates. Those with Ki67

AIM: To determine whether callosal angle (CA) measurement, a diagnostic and prognostic tool used for normal-pressure hydrocephalus in adults, is a reliable radiological parameter for evaluating endoscopic third ventriculostomy (ETV) outcomes in pediatric patients. MATERIAL and METHODS: Forty-seven pediatric patients with hydrocephalus who underwent successful EN in our clinic between 2011 and 2015 were included in this study. Preoperative and postoperative three-month CA, lateral ventricle frontal horn (LVFH) width, Evans' index (El), and frontal-occipital horn ratio (FOR) parameters were recorded, with changes analyzed using a paired-samples t-test. RESULTS: There were 29 male and 18 female patients included within the cohort. For mean preoperative values, LVFH width was 58.8 +/- 14.9 mm, El was 0.43 +/- 0.09, FOR was 0.51 +/- 0.74, and CA was 78.5 degrees +/- 36.4 degrees. Separately, for mean postoperative values, LVFH width was 54 +/- 14.2 mm, El was 0.39 +/- 0.09, FOR was 0.47 +/- 0.07, and CA was 104.5 degrees +/- 32.6 degrees. The CA was increased and the LVFH width, El, and FOR were decreased in all patients within three months after surgery. The postoperative three-month change in CA was higher than those observed in the other parameters. CONCLUSION: Changes in CA after successful EN were dramatically higher than those in the other ventricular parameters. For this reason, we suggest CA be used as a radiological criterion during early radiological follow-up of patients after ETV.

Mature pineal region teratomas differ from other pineal tumors in terms of their characteristic radiological appearance and their clinical outcome after gross total excision. Our aim is to share our clinical experience and treatment outcomes in pediatric patients with mature pineal region teratoma. In this retrospective study, we reviewed clinical, radiological, and surgical data of ten patients who had radiologically predicted diagnosis of pineal region teratoma and pathologically confirmed diagnosis of mature pineal region teratoma between years 2004 and 2017 in our clinic. Statistical analysis was performed with SPSS 20. All patients were male. Ages of patients ranged between 5 and 17 (median age was 9.5). All of them presented with headache. Magnetic resonance imaging showed characteristic appearance of teratomas with variable degree of hydrocephalus. All patients had negative results for AFP and b-HCG levels. All patients had gross total resection of pineal tumor through occipital transtentorial approach with no permanent neurological deficit. Pathological results of all tumor samples were consistent with mature teratoma. None of them had adjuvant chemotherapy or radiotherapy. Follow-up periods ranged between 3 and 170 months (median follow-up period was 60.5 months). All patients are alive with no tumor recurrence. Pediatric mature pineal region teratomas are benign tumors with characteristic MRI appearance and negative tumor markers. Their definitive treatment is gross total surgical excision. Occipital transtentorial approach is a safe procedure for treatment of pediatric mature pineal teratomas.

Metastasis of an intracranial tumour is not a common situation in our daily neurosurgical practice. Pleomorphic xanthoastrocytoma is also a rare glial tumour with relatively a favourable prognosis among other CNS pathologies. Here, we present an anaplastic pleomorphic xanthoastrocytoma case which shows both haematogenous and lymphatic metastasis which is described first time in the up-to-date literature. Our case is a 17-year-old male operated for a right occipital intra-axial lesion with a diagnosis of anaplastic pleomorphic xanthoastrocytoma which recurs 5 years later and metastasize vigorously through haematogenous and lymphatic routes. A rare-presenting symptom for this pathology is also intracerebral haemorrhage. This is the ninth case report in the literature which presents initially with this entity.

AIM: Vagal nerve stimulation (VNS) is an effective method of treatment for epilepsy patients either unresponsive to medical therapy or not suitable for resective surgeries. We designed an experimental study on Wistar Albino Glaxo rats from Rijswijk (WAG\textbackslash{}Rij) to investigate the effects of VNS on a non-convulsive epilepsy model. MATERIAL and METHODS: The experiment was performed on six WAG/Rij rats, a validated strain for genetic absence seizures. The animals were underwent VNS and the effects were investigated on electroencephalography (EEG) recordings at 22, 24, 26 hours of stimulation and 15 days after the cessation, for duration of spike and wave complexes (SWC), the numbers, mean duration of SWC and frequencies in an hour. RESULTS: EEG recordings demonstrated that the mean duration of SWC was 353.1 seconds and the number of activity per hour was 62 at the baseline. There were statistically significant decreases in the total duration of SWC and the number of activities (61.8\% and 78\% decrease, respectively). There were no significant decreases in the mean duration of SWC and the frequencies. CONCLUSION: The acute stimulation of the vagal nerve caused a statistically significant decrease both in overall duration of SWC and the number of complexes in an hour. Moreover, the positive effects seemed to last even 15 days after the cessation of the stimulation. Further studies focusing on different stimulation parameters and delayed effects of the VNS on human absence seizures are warranted.

The role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS).A total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI.Nineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI.Abnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD.

The aim is to describe the behavior of pilocytic astrocytoma (PAs) and its effects on patient prognosis by using flow cytometric, immunohistochemical and cytogenetic methods. We also aim to find out whether there is any difference between differently localized tumors by the above mentioned analyses. We studied DNA index, expression of p53, p16, pRb, MMAC/PTEN1, VEGF, MIB-1 index and chromosomal anomalies which can be detected by array comparative genomic hybridization (CGH) technique. We analyzed the association of the results of these studies with clinical prognosis and tumor localization. We included 53 patients (18 cerebellar, 20 chiasmatic/hypothalamic and 15 hemispheric). Samples were studied from paraffin embedded tumors. We found that PAs are mostly diploid and ploidy pattern does not affect the prognosis. The expression of p53, p16, pRb, MMAC/PTEN1 and VEGF was not significantly different between different localizations and could not predict the prognosis. Frequently seen copy number aberrations (CNAs) are: amplification in 1p36.33, 2p11.2, 9p11.2, 9q12, 16p11.2, 19q13.12–q13.2, Xp22.2–p21.3, Xp11.3–p11.22, Xq11.1–q12, Xq13.1, Xq21.1–q21.31, Xq22.3, Xq26.3 and homozygous deletion in 2p11.2, 8p23.1, 16p12.3. Among them, 2p11.2 amp, 9p11.2 amp and 1p36.21 hom del were correlated with prognosis. Moreover, we found a significant correlation between 16p11.2 amp and tumor localization. Differently localized PAs have different properties which make them behave with different biological aggressiveness. PAs demonstrate a significant amount of CNAs that can be detected by a high-resolution study. However, tumor suppressor genes p53, p16, pRb, MMAC/PTEN1 and expression patterns do not play a significant role in PAs.

Background: The aim of this study was to determine electrocardiographic changes in children during seizures. Methods: We assessed heart rate changes, RR intervals and QT changes during 47 seizures in children. Consecutive QT and RR intervals were measured for 60 s before the seizures, during the seizures and 60 s after the seizures during video electroencephalography monitoring. Results: There were 47 seizures in 18 patients. Five patients had generalized seizures and 13 patients had focal seizures. Twelve patients were male. The mean age during monitoring was 10.1 years (range 4 months–19 years). Ictal tachycardia was seen in every seizure. No ictal bradycardia was noted. There was only one dropped beat in a patient. The mean ictal heart-rate-corrected QT (QTc) interval was significantly higher than the postictal measurements (P= 0.005). Mean ictal QTc variation tended to increase during seizures and then decreased below the pre-ictal measurements in the postictal period. However these changes were statistically insignificant (P > 0.05). RR variance was significantly decreased during seizures compared to the postictal period and the standard deviation of the RR intervals was significantly decreased in the ictal period compared to the pre-ictal period (P= 0.014 and P= 0.001, respectively). Conclusion: Tachycardia is the main finding in seizures in children. Ictal bradycardia and cardiac arrhythmias are very rare despite being more frequent in adults with seizures.

The aim of this review is to present the contemporary role of radiology in evaluating pediatric hydrocephalus. Although conventional brain imaging with ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) reveal the degree of ventricular enlargement and often the etiology of the hydrocephalus, the diagnosis and management of hydrocephalus present common problems in pediatric populations.US, usually sufficient to assess and monitor ventricular size, is used most commonly in preterm infants who have germinal matrix hemorrhages and not able to tolerate transport to the radiology department. Although CT can demonstrate gross dilatation of ventricles, in most cases, it will be necessary to more closely define the nature of the obstruction, either functionally or anatomically. MRI is the best imaging modality to provide such functional and anatomic information. However, since identification of obstructive pathologic processes at any level through the cerebrospinal fluid (CSF) pathway in patients with hydrocephalus is of significant importance because it can change the treatment options, avoiding shunt insertion, a more sophisticated MRI approach is needed instead of obtaining a routine cranial MRI. Furthermore, the outcome after neuroendoscopic procedures is clearly related to patient selection under guidance of neuroimaging.Therefore, the article focuses mainly on the effective usage of various MRI sequences in both diagnosis and follow-up of pediatric hydrocephalus, such as 3D CISS, cine PC, TSE, and GRE T2* sequences, to be able to investigate all possible obstructive pathology through the CSF pathway and to assess the efficiency of treatment in a standardized way.

Object The authors describe the first shared-resource, 3-T intraoperative MR (ioMR) imaging system and analyze its impact on low-grade glioma (LGG) resection with an emphasis on the use of intraoperative proton MR spectroscopy. Methods The Acibadem University ioMR imaging facility houses a 3-T Siemens Trio system and consists of interconnected but independent MR imaging and surgical suites. Neurosurgery is performed using regular ferromagnetic equipment, and a patient can be transferred to the ioMR imaging system within 1.5 minutes by using a floating table. The ioMR imaging protocol takes < 10 minutes including the transfer, and the authors obtain very high–resolution T2-weighted MR images without the use of intravenous contrast. Functional sequences are performed when needed. A new 5-pin headrest–head coil combination and floating transfer table were specifically designed for this system. Results Since the facility became operational in June 2004, 56 LGG resections have been performed using ioMR imaging, and > 19,000 outpatient MR imaging procedures have been conducted. First-look MR imaging studies led to further resection attempts in 37.5% of cases as well as a 32.3% increase in the number of gross-total resections. Intraoperative ultrasonography detected 16% of the tumor remnants. Intraoperative proton MR spectroscopy and diffusion weighted MR imaging were used to differentiate residual tumor tissue from peritumoral parenchymal changes. Functional and diffusion tensor MR imaging sequences were used both pre- and postoperatively but not intraoperatively. No infections or other procedure-related complications were encountered. Conclusions This novel, shared-resource, ultrahigh-field, 3-T ioMR imaging system is a cost-effective means of affording a highly capable ioMR imaging system and increases the efficiency of LGG resections.

BACKGROUND AND PURPOSE: 3D-constructive interference in steady state (3D-CISS) sequence has been used to assess the CSF pathways. The aim of this study was to investigate the additive value of 3D-CISS compared with conventional sequences in the diagnosis of obstructive membranes in hydrocephalus. MATERIALS AND METHODS: A total of 134 patients with hydrocephalus underwent MR imaging examination with a 3T unit consisting of turbo spin-echo, 3D-CISS, and cine phase-contrast (cine PC) sequences. 3D-CISS was used to assess obstructive membranes in CSF pathways compared with other sequences. Cine PC, follow-up imaging, and surgical findings were used to confirm obstructive membranes. RESULTS: Comparing the number of noncommunicating cases by using the conventional and 3D-CISS images, we found 26 new cases (19.4%) of 134 cases that were previously misdiagnosed as communicating hydrocephalus by conventional images. 3D-CISS sequence identified obstructive membranes invisible in other sequences, which facilitated selection of neuroendoscopy in the treatment of 31 patients (23.1%) in total who would have been otherwise treated with shunt insertion. These patients included 26 newly diagnosed noncommunicating cases after demonstration of intraventricular and/or fourth ventricular outlet membranes and 5 cases of communicating hydrocephalus with obstructing cisternal membranes. There were obstructions of the foramina of Luschka in 22 of 26 newly found noncommunicating cases. CONCLUSIONS: Conventional sequences are insensitive to obstructive membranes in CSF pathways, especially in the fourth ventricular exit foramina and the basal cisterns. 3D-CISS sequence, revealing these obstructive membranes, can alter patient treatment and prognosis.

In children with spasticity, deep tendon reflexes are hyperactive and even stimulation of normal dorsal rootlets can produce exaggerated full-strength, single-twitch responses in the muscles they innervate. This phenomenon is called the brisk hyperactive response (BHR). The aim of this study was to compare the efficacy of 2 volatile anesthetics, isoflurane and sevoflurane, for suppressing the confounding effect of BHR during selective dorsal rhizotomy (SDR) in children with spasticity. The subjects were 54 consecutive children of American Society of Anesthesiology physical status III who were scheduled for SDR. After tracheal intubation, each child was randomly assigned to Group I (isoflurane; n = 27) or Group S (sevoflurane; n = 27). There was no significant difference between the mean operation times in Groups I and S (200 ± 40 vs. 220 ± 35 minutes, respectively; p = 0.0559). Thirteen patients in Group I (48.1%) and 5 in Group S (18.5%) exhibited BHR during stimulation of the dorsal rootlets (odds ratio 4.086; p = 0.0418). Three of these 18 patients (2 in Group I and 1 in Group S) experienced hypertension and tachycardia simultaneously with BHR (odds ratio 4.086; p = 1.0). The results suggest that sevoflurane is more effective at preventing BHR and might be a better choice for anesthetic management of children with spasticity undergoing SDR.

✓ Primary meningeal osteosarcomas are rare tumors, with only 19 reported cases in the literature; only 4 of these, including the present case, are in pediatric patients. In this report, the authors present the case of an 8-year-old boy with a history of generalized tonic–clonic seizures who was found to harbor a meningeal osteosarcoma within the sylvian fissure. Initial working diagnoses included meningioma and glioma. After tumor enlargement and progressive symptoms, the patient underwent a large frontotemporal craniotomy and complete resection of the lesion, which recurred 6 and 12 months after the initial surgery and was surgically treated after each recurrence. The rarity of primary meningeal osteosarcomas can make their diagnosis difficult, and histopathological evaluation is mandatory for diagnosis. Because of their fast progression, they must be treated aggressively by means of surgery, chemotherapy, and radiotherapy.

Schilder’s myelinoclastic diffuse sclerosis (Schilder’s disease) is a rare demyelinating disorder. Clinical features and neuroimaging findings of this disease might mimic an intra-cranial mass lesion including an abscess or a tumour. Clinical and radiological findings of two children with the diagnosis of Shilder’s disease are reported, and the role of brain biopsy as a diagnostic tool is discussed.

Suprasellar arachnoid cysts are rare lesions that constitute approximately 5-12.5% of all arachnoid cysts. We present a male infant diagnosed in utero with suprasellar arachnoid cyst and treated with endoscopic ventriculocystocisternotomy on the 38th day of life. The patient developed hyponatremia as a result of cerebral salt wasting after the operation.

Lumbar disc herniation very rarely occurs in adolescence. The aim of this study was to assess the radiological, clinical and surgical features and case outcomes for adolescents with lumbar disc herniation, and to compare with adult cases. The cases of 17 adolescents (7 girls and 10 boys, age range 13–17 years) who were surgically treated for lumbar disc herniation in our clinic between 1998 and 2003 were retrospectively reviewed. The mean follow-up time for these cases was 60 months. The collected histories revealed that 14 (82%) of the 17 cases involved trauma or intense sports activity. Low back pain was the most common complaint (15 cases, 88%). None of the 17 patients had major symptoms during follow-up, and most were engaged in intense sports or heavy work-related activities during this period. The main features of lumbar disc herniation in adolescents are different from those seen in adults. Careful assessment is vital to avoid misdiagnosis and prevent undesirable results from inappropriate surgery. When surgery is indicated and patients are carefully selected, the results of lumbar discectomy in adolescence can be satisfactory.

Primary brain stem tumors are a group of diseases that may be located in the mesencephalon, pons, and medulla oblongata. They are generally considered as diseases with a poor prognosis, because surgery nearby such eloquent structures within the brain stem is a great challenge for many neurosurgeons and complete resection can be achieved in only a small number of selected patients.