Surgical management of lobar cerebral cavernous malformations in children: a single-center experience.

PURPOSE: We aimed to determine the surgical indications and postoperative outcomes among pediatric patients with lobar cerebral cavernous malformations (CCMs). METHODS: We retrospectively reviewed pediatric patients operated on for lobar CCM between March 2010 and August 2021. Indications for surgery included (1) intracranial hemorrhage, (2) symptomatic superficially located lesion, and (3) asymptomatic CCM in non-eloquent area in case of strong parental preferences. Patients presenting with seizures were assessed using Engel Epilepsy Surgery Outcome Scale. RESULTS: Twenty-one patients were included. The predominant symptoms were seizures (57.1%), headaches (33.3%), and focal neurological deficits (23.8%). Patients were qualified for surgery due to symptomatic intracranial hemorrhage (47.6%), drug-resistant epilepsy (28.6%), and focal neurological deficits (9.5%). Three patients (14.3%) were asymptomatic. A gross total resection of CCM with the surrounding hemosiderin rim was achieved in all patients. The mean follow-up was 52 months. No patient experienced surgery-related complications. In all individuals with a preoperative first episode of seizures or focal neurological deficits, the symptoms subsided. All six patients with drug-resistant epilepsy improved to Engel classes I (67%) and II (33%). CONCLUSION: Surgical removal of symptomatic lobar CCMs in properly selected candidates remains a safe option. Parental preferences may be considered a sole qualifying criterion for asymptomatic lobar CCM excision.

Open Fetal Surgery for Ventricular-Amniotic Valve Implantation in Aqueductal Stenosis-Dependent Severe Fetal Hydrocephalus: A Case Report with 7-Year Follow-Up.

INTRODUCTION: Fetal ventriculomegaly is one of the most commonly diagnosed central nervous system pathologies of the second trimester, occurring with a frequency of 0.3-0.5/1,000 births. Severe fetal ventriculomegaly (SVM) may necessitate intrauterine intervention. Most such interventions have been made percutaneously with ultrasound guidance insertion of a pigtail catheter, which sadly often became obstructed or migrated. CASE PRESENTATION: Our case report presents the possibility of ventriculo-amniotic valve implantation (VAVI) by classic hysterotomy in isolated severe fetal hydrocephalus (IVSM) due to aqueductal stenosis. The patient was operated on similarly to open fetal surgery MOMS criteria at 24+4/7 GA, with an initial lateral ventricular dimension of 22.5 mm. A female newborn was delivered by elective cesarean section at 31+1/7 GA due to PPROM (Apgar 10' 8 points, birth weight 1,600 g), required CPAP, and removal of the drainage system due to infection and narrow lateral ventricles. Evans index (EI) gradual increase and clinical symptoms of high-pressure hydrocephalus after 10 days required a ventricle-peritoneal shunt (VPS) implantation. The newborn was discharged home after 28 days with stabile hydrocephalus (EI: 0.59-0.6), in good clinical condition. The 7-year follow-up was complicated by epilepsy, VPS shunt infections, delay in motor and intellectual functions (mild to moderate), and symptoms of atypical autism, the phenotype possibly related to a variant in ZEB2 gene. CONCLUSION: Intrauterine VAVI is a one-step procedure that is effective in draining CFS. The limitations of the method remain complications due to preterm labor and infection of the drainage system.

External validation of the Chicago Chiari Outcome Scale in adults with Chiari malformation type I.

OBJECTIVE: The Chicago Chiari Outcome Scale (CCOS) serves as a standardized clinical outcome evaluation tool among patients with Chiari malformation type I (CM-I). While the reliability of this scale has been proven for pediatric patients, the literature lacks CCOS validation when used solely in adults. Therefore, this study aimed to determine the validity of the CCOS in an external cohort of adult patients. METHODS: The authors retrospectively analyzed the medical records of symptomatic patients with CM-I who underwent posterior fossa decompression between 2010 and 2018 in six neurosurgical departments. Each patient was clinically assessed at the latest available follow-up. Gestalt outcome was determined as improved, unchanged, or worsened compared with the preoperative clinical state. Additionally, the CCOS score was calculated for each patient based on the detailed clinical data. To verify the ability of the CCOS to determine clinical improvement, the area under the receiver operating characteristic (AUROC) curve was evaluated. A logistic regression analysis using all four components of the CCOS (pain symptoms, nonpain symptoms, functionality, and complications) was performed to establish predictors of the improved outcome. RESULTS: Seventy-five individuals with a mean age of 42 ± 15.32 years were included in the study. The mean follow-up duration was 52 ± 33.83 months. Considering gestalt outcome evaluation, 41 patients (54.7%) were classified as improved, 24 (32%) as unchanged, and 10 (13.3%) as worsened. All patients with a CCOS score of 14 or higher improved, while all those with a CCOS score of 8 or lower worsened. The AUROC was 0.986, suggesting almost perfect accuracy of the CCOS in delineating clinical improvement. A CCOS score of 13 showed high sensitivity (0.93) and specificity (0.97) for identifying patients with clinical improvement. Additionally, a meaningful correlation was found between higher CCOS scores in each component and better outcomes. Patient stratification by total CCOS score showed that those categorized as improved, unchanged, and worsened scored prevalently between 13 and 16 points, 10 and 12 points, and 4 and 9 points, respectively. CONCLUSIONS: In this adult cohort, the CCOS was found to be almost perfectly accurate in reflecting postoperative clinical improvement. Moreover, all four CCOS components (pain symptoms, nonpain symptoms, functionality, and complications) significantly correlated with patient clinical outcomes.

External validation of the Ruptured Arteriovenous Malformation Grading Scale (RAGS) in a multicenter adult cohort.

PURPOSE: While Ruptured Arteriovenous Malformation Grading Scale (RAGS) has recently been validated in children, the literature lacks validation on adults exclusively. Therefore, we aimed to determine the validity of RAGS on the external multicenter adult cohort and compare its accuracy with other scales. METHODS: A retrospective analysis was performed in five neurosurgical departments to extract patients who presented with the first episode of acute brain arteriovenous malformation (bAVM) rupture between 2012 and 2019. Standard logistic regression and area under the receiver operating curve (AUROC) calculations were performed to determine the value of the following scales: intracerebral hemorrhage (ICH), AVM-associated ICH (AVICH), Spetzler-Martin (SM), Supplemented SM (Supp-SM), Hunt and Hess (HH), Glasgow Coma Scale (GCS), World Federation of Neurological Surgeons (WFNS), and RAGS to predict change in categorical and dichotomized modified Rankin Scale (mRS) across three follow-up periods: within the 6 months, 6 months to 1 year, and above 1 year. RESULTS: Sixty-one individuals with a mean age of 43.6 years were included. The RAGS outperformed other grading scales during all follow-up time frames. It showed AUROC of 0.78, 0.74, and 0.71 at the first 6 months, between 6 and 12 months, and after 12 months of follow-up, respectively, when categorized mRS was applied, while corresponding values were 0.79, 0.76, and 0.73 for dichotomized mRS, respectively. CONCLUSION: The RAGS constitutes a reliable scale predicting clinical outcomes following bAVM rupture among adults. Furthermore, the RAGS proved its generalizability across medical centers with varying treatment preferences.

Clinical application of diffusion tensor imaging and fiber tractography in the management of brainstem cavernous malformations: a systematic review.

This study aimed to systematically review the literature to determine the clinical utility and perspectives of diffusion tensor imaging (DTI) in the management of patients with brainstem cavernous malformations (BSCMs). PubMed, Embase, and Cochrane were searched for English-language articles published until May 10, 2021. Clinical studies and case series describing DTI-based evaluation of patients with BSCMs were included. Fourteen articles were included. Preoperative DTI enabled to adjust the surgical approach and choose a brainstem safe entry zone in deep-seated BSCMs. Preoperatively lower fractional anisotropy (FA) of the corticospinal tract (CST) correlated with the severity of CST injury and motor deficits. Postoperatively increased FA and decreased apparent diffusion coefficient (ADC) corresponded with the normalization of the perilesional CST, indicating motor improvement. The positive (PPV) and negative predictive value (NPV) of qualitative DTI ranged from 20 to 75% and from 66.6 to 100%, respectively. The presence of preoperative and postoperative motor deficits was associated with a higher preoperative resting motor threshold (RMT) and lower FA. A higher preoperative CST score was indicative of a lower preoperative and follow-up Medical Research Council (MRC) grade. DTI facilitated the determination of a surgical trajectory with minimized risk of WMTs' damage. Preoperative FA and RMT might indicate the severity of preoperative and postoperative motor deficits. Preoperative CST score can reliably reflect patients' preoperative and follow-up motor status. Due to high NPV, normal CST morphology might predict intact neurological outcomes. Contrarily, sparse and relatively low PPV limits the reliable prediction of neurological deficits.

Relevance of intraoperative motor evoked potentials and D-wave monitoring for the resection of intramedullary spinal cord tumors in children.

The purpose of this study was to evaluate the reliability of motor evoked potentials (MEP), somatosensory evoked potentials (SSEP), and D-wave monitoring as predictors of postoperative neurological deficits in pediatric patients undergoing resection of intramedullary spinal cord tumors (IMSCTs). Additionally, we aimed to determine whether alerts in the intraoperative neurophysiological monitoring (IONM) influenced the extent of resection (EOR). A retrospective analysis of the pediatric patients who underwent resection of IMSCT between March 2010 and April 2021 with an IONM guidance was performed. IONM alerts were recorded and correlated with patients' clinical status at discharge. Twenty three pediatric patients were included. MEP and SSEP were successfully elicited in all patients, while D-wave monitoring was feasible for 14 of them (60.9%). Significant IONM alerts occurred in 6 individuals (26.1%) with monitorable MEP and SSEP and 2 patients with monitorable D-waves (14.3%). The sensitivity, specificity, positive predictive value, and negative predictive value accounted for 100%, 81.8%, 20%, 100% for MEP, 100%, 92.3%, 50%, 100% for D-wave, and 50%, 81%, 20%, 94.44% for SSEP, respectively. Both MEP (p < 0.001) and D-wave monitoring (p < 0.001) accurately predicted postoperative motor deficits, while SSEP failed to provide significant accuracy regarding sensory deficits (p = 0.491). Gross-total tumor resection was performed in 29.4% of patients without IONM alerts and 33.3% of patients with IONM alerts, indicating that IONM alerts did not limit the EOR (p = 0.0857). MEP and D-wave monitoring can be perceived as reliable IONM modalities in pediatric IMSCTs surgery. Caution is needed with the implementation of SSEP to guide surgical decisions.

Subependymal giant cell astrocytomas are characterized by mTORC1 hyperactivation, a very low somatic mutation rate, and a unique gene expression profile.

Subependymal giant-cell astrocytomas (SEGAs) are slow-growing brain tumors that are a hallmark feature seen in 5-10% of patients with Tuberous Sclerosis Complex (TSC). Though histologically benign, they can cause serious neurologic symptoms, leading to death if untreated. SEGAs consistently show biallelic loss of TSC1 or TSC2. Herein, we aimed to define other somatic events beyond TSC1/TSC2 loss and identify potential transcriptional drivers that contribute to SEGA formation. Paired tumor-normal whole-exome sequencing was performed on 21 resected SEGAs from 20 TSC patients. Pathogenic variants in TSC1/TSC2 were identified in 19/21 (90%) SEGAs. Copy neutral loss of heterozygosity (size range: 2.2-46 Mb) was seen in 76% (16/21) of SEGAs (44% chr9q and 56% chr16p). An average of 1.4 other somatic variants (range 0-7) per tumor were identified, unlikely of pathogenic significance. Whole transcriptome RNA-sequencing analyses revealed 190 common differentially expressed genes in SEGA (n = 16, 13 from a prior study) in pairwise comparison to each of: low grade diffuse gliomas (n = 530) and glioblastoma (n = 171) from The Cancer Genome Atlas (TCGA) consortium, ganglioglioma (n = 10), TSC cortical tubers (n = 15), and multiple normal tissues. Among these, homeobox transcription factors (TFs) HMX3, HMX2, VAX1, SIX3; and TFs IRF6 and EOMES were all expressed >12-fold higher in SEGAs (FDR/q-value < 0.05). Immunohistochemistry supported the specificity of IRF6, VAX1, SIX3 for SEGAs in comparison to other tumor entities and normal brain. We conclude that SEGAs have an extremely low somatic mutation rate, suggesting that TSC1/TSC2 loss is sufficient to drive tumor growth. The unique and highly expressed SEGA-specific TFs likely reflect the neuroepithelial cell of origin, and may also contribute to the transcriptional and epigenetic state that enables SEGA growth following two-hit loss of TSC1 or TSC2 and mTORC1 activation.

Clinical features, microbiology, and management of pediatric brainstem abscess.

PURPOSE: Brainstem abscess is a rare condition accounting for merely 1% of brain abscesses incidence in the pediatric population. This study aimed to present a single patient with a pontine abscess and review the literature to highlight clinical features, diagnosis, and management of brainstem abscess. METHODS: The PubMed database was screened for English-language articles concerning pediatric brainstem abscess. We, therefore, identified 22 publications, which concisely depict 23 cases. Our study reports on the 24th pediatric patient diagnosed with that entity. All included reports were analyzed in terms of clinical presentation, diagnosis, management, and outcomes of described patients. RESULTS: There was slight women predominance (15:9), with a mean age of occurrence 6.4 years, ranging from 7 months to 16 years. Pons was the most common location of brainstem abscess, occurring in 75% of patients. Clinically, they mostly presented with cranial nerves palsy (79.2%), hemiparesis (66.7%), and pyramidal signs (45.8%). The classic triad of symptoms, including fever, headache, and the focal neurologic deficit was present in 20.8% of patients. Positive pus cultures were obtained in 61.1%. Streptococci and Staphylococci were the most frequently identified pus microorganisms. Outcomes were satisfactory, with a 79.2% rate of general improvement. CONCLUSIONS: Neurosurgical aspiration is a safe and beneficial therapeutic method. It should always be considered and should promptly be performed when the conservative treatment is not successful and clinical deterioration occurs. Prognosis in pediatric brainstem abscess is generally favorable. Most patients recover with minor neurologic deficits or improve completely.

Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS).

PURPOSE: The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. METHODS: We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC z-score, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. RESULTS: Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up. CONCLUSION: ETV and shunting led to improvements in HC centile, z-score, and FOR measurements during long-term follow-up of infants with hydrocephalus secondary to aqueductal stenosis. Head size did not significantly differ between the treatment groups during follow-up, however ventricle size was greater in those undergoing ETV when measured at 1 and 3 years following treatment.

Radiosurgery or hypofractionated stereotactic radiotherapy after craniospinal irradiation in children and adults with medulloblastoma and ependymoma.

PURPOSE: To assess the results and tolerance of radiosurgery/hypofractionated stereotactic radiotherapy performed after craniospinal irradiation for recurrent tumor. METHODS: Fourteen patients aged 3-46 years, diagnosed with medulloblastoma (10), anaplastic ependymoma (3), and primitive neuroectodermal tumor (1). All patients had craniospinal irradiation (CSI) with the total dose of 30.6-36 Gy and boost to 53.9-60 Gy either during primary or during second-line treatment. Twelve patients were irradiated with a single dose of 6-15 Gy (median 14.5 Gy). One received three fractions of 5 Gy and one six fractions of 5 Gy. In statistical analysis, the Kaplan-Meier method and log-rank test were used. The overall survival was calculated from the date of the end of stereotactic radiosurgery to the date of death or last contact. RESULTS: Recurrences were diagnosed after the median time of 16 months after the end of primary treatment. Eleven patients died during the follow-up. The follow-up for the 3 patients still alive was 6.7, 40.5, and 41.4 months, respectively. One- and 2-year overall survival (OS) was 70% and 39%. Patients who had ECOG performance status of 0 at the time of diagnosis of the disease trended to have better 2-year OS compared to those evaluated as ECOG 1 (p = 0.057). Treatment results were evaluable in 12 patients. Local control (stabilization or regression of the lesion) was achieved in 9 (75%). Overall disease progression was 67%. No patient developed radiation-induced necrosis. The treatment was well tolerated and no serious adverse effects were observed. Eleven patients were given steroids as a prevention of brain edema and four of them needed continuation of this treatment afterwards. In 7 patients, symptoms of brain edema were observed during the first weeks after reirradiation. CONCLUSIONS: Stereotactic radiosurgery or hypofractionated stereotactic radiotherapy is an effective treatment method of the local recurrence after CSI and can be performed safely in heavily pre-treated patients.

Results of the surgical treatment in children with Chiari malformation type I.

PURPOSE: Our study aimed to evaluate the quality of life of the patients operated due to Chiari malformation type 1 (CM-1) in the Department of Pediatric Neurosurgery, Medical University of Silesia in Katowice. METHODS: We performed a retrospective analysis of 11 patients diagnosed with CM-1 who were treated in our center in the years 2007 to 2016. There were 6 female and 5 male individuals. Short-term evaluation of the outcome was based on comparison of the presenting symptoms and radiological images before and after the surgical treatment. Long-term follow-up was carried out using survey questionnaires based on the Chicago Chiari Outcome Scale (CCOS) devised originally by Aliaga et al. RESULTS: Patients, based on their CCOS score were divided into three groups marked as "improved," "unchanged," and "worse," depending on a range of CCOS score: 13-16, 9-12, 4-8, respectively. The outcome of patients was as follows: 6 patients (55%) were evaluated as improved, and 5 (45%) as unchanged. No patient was classified as worse after surgery. Significant negative Spearman's correlation was found between the CCOS score and patients' age at the time of surgery (R = - 0.85, p = 0.0009). CONCLUSIONS: The decision of whether to operate pediatric patients with CM-1 should be considered very carefully. In our department, the main indication for surgery was the occurrence of clinical symptoms. Our study revealed that in the symptomatic patients, surgery improves their quality of life measured with CCOS.

The components of shoulder and elbow movements as goals of primary reconstructive operation in obstetric brachial plexus lesions.

Most of the cases of obstetric brachial plexus lesions (OBPL) show satisfactory improvement with conservative management, but in about 25% some surgical treatment is indicated. The present paper analyzes the effects of primary reconstructive surgeries in aspect of achieving delineated intraoperatively goals. Children operated before the age of 18 months with follow-up period longer than 1 year were selected. Therapeutic goals established during the operation were identified by analysis of initial clinical status and operative protocols. The elementary movement components in shoulder and elbow joints were classified by assessing range of motion, score in Active Movement Scale and modified British Medical Research Council scale of muscle strength. The effect was considered satisfactory when some antigravity movement was possible, and good when strength exceeded M3 or antigravity movement exceeded half of range of passive movement. In 13 of 19 patients most of established goals were achieved at good level, in 2 at satisfactory level. Remaining 4 patients showed improvement only in some aspects of extremity function. In 2 patients improvement in some movements was accompanied by worsening of other movements. The analysis of results separated into individual components of movements showed that goals were achieved in most of the cases, simultaneously clearly indicating which damaged structures failed to provide satisfactory function despite being addressed intraoperatively. The good results were obtained mainly by regeneration through grafts implanted after resection of neuroma in continuity, which proves that this technique is safe in spite of unavoidable temporary regression of function postoperatively.

Infratentorial ependymomas--a study of the centre in Katowice.

The aim of the study was to assess the correlation of the results of the treatment of infratentorial ependymomas with the degree of resection and histopathological diagnosis. The study was conducted on a group of 19 patients, 13 boys and 6 girls aged 3 months to 16 years, with infratentorial ependymoma treated at the Department of Paediatric Neurosurgery of the Medical University of Silesia in Katowice from January 2000 until December 2008. The most significant factor having an impact on overall survival and progression-free survival was totality of tumour resection. There has been no statistically significant influence of the histopathological type of ependymoma on the result of treatment. The tendency to report better results of treatment of non-anaplastic ependymoma seems to derive from a statistically higher frequency of total removal of tumours of this type.

Radiological findings in relation to the neurodevelopmental outcome in hydrocephalic children treated with shunt insertion or endoscopic third ventriculostomy.

PURPOSE: The aim of the study was to compare the radiological indicators of effectiveness for hydrocephalus treatment in children operated on under the third year of age with the use of shunt insertion (SI) and endoscopic third ventriculostomy (ETV). The effectiveness was considered in terms of postoperative neurodevelopment in correlation to pre- and postoperative radiological findings. METHODS: The examined group consisted of 46 children operated on for hydrocephalus in the Division of Pediatric Neurosurgery in Katowice, Poland. There were 21 children treated with SI and 25 with ETV. The radiographic assessment was carried out on the basis of MRI and CT examinations with the use of a linear estimate known as frontal and occipital horn ratio (FOR). The FOR values were calculated for the entire group and in correlation to the treatment method and to the children neurodevelopment evaluated with The Denver Developmental Screening Test. RESULTS: No differences were recognized between initial FOR value in terms of the postoperative children neurodevelopment. In the successful ETV-treated subgroup, the mean change in FOR was 0.05 and in the SI-treated subgroup, the mean change in FOR 0.13. The patients with BFOR >0.1, developed normally more often than those in whom BFOR was lower than 0.1. CONCLUSIONS: The initial FOR value probably does not affect the postoperative developmental outcome. Long-term change in ventricles size after surgery can correlate with psychomotor development of hydrocephalic children. Presumably, there are no differences between two treatment options according to initial FOR values and to changes in FOR values.

Congenital subependymal giant cell astrocytomas in patients with tuberous sclerosis complex.

PURPOSE: Subependymal giant cell astrocytoma (SEGA) is a brain tumor associated with tuberous sclerosis complex (TSC). It usually grows in a second decade of life, but may develop in the first months of life. The aim of this work was to establish the incidence, clinical features, and outcome of congenital SEGA in TSC patients. METHODS: Cohort of 452 TSC patients was reviewed to identify cases with growing or hydrocephalus producing SEGAs in the first 3 months of life. Clinical presentation, size of the tumor, growth rate, mutational analysis, treatment applied, and outcome were analyzed. RESULTS: Ten (2.2 %) patients presented with SEGA in the first 3 months of life. All of them had documented SEGA growth and all developed hydrocephalus. In eight patients, mutational analysis was done, and in all of them, TSC2 gene mutations were identified. Mean maximum SEGA diameter at baseline was 21.8 mm. Mean SEGA growth rate observed postnatally was 2.78 mm per month and tended to be higher (5.43 mm per month) in patients with TSC2/PKD1 mutation than in other cases. Seven patients underwent SEGA surgery and surgery-related complications were observed in 57.1 % cases. One patient was successfully treated with everolimus as a primary treatment. CONCLUSIONS: Congenital SEGA develops 2.2 % of TSC patients. Patients with TSC2 mutations, and especially with TSC2/PKD1 mutations, are more prone to develop SEGA earlier in childhood and should be screened for SEGA from birth. In young infants with SEGA, both surgery and mTOR inhibitor should be considered as a treatment option.

The Price of Success-The Long-Term Outcomes of Children with Craniopharyngioma-Two Institutions' Experience.

An analysis of patients below 21 years old treated due to craniopharyngioma in the years 1979-2022 was performed with the aim of evaluating the long-term outcome and treatment side-effects. The standard statistical tests were used, and 56 patients with a median age of 11 years were evaluated. Surgery was the primary treatment in 55 patients; however, in only 29 it was the only neurosurgical intervention. Eighteen children were treated with radiotherapy (RTH) in primary treatment. The most common neurosurgical side effects observed were visual and endocrine deficits and obesity, which were diagnosed in 27 (49%), 50 (91%), and 25 (52%) patients, respectively. Complications after RTH were diagnosed in 14 cases (32%). During the median follow-up of 8.4 years (range: 0.4-39.8 years), six patients died and the 5- and 10-year overall survival was 97% and 93%, respectively. Five-year progression-free survival for gross total resection, resection with adjuvant RTH, and non-radical resection alone was 83%, 68%, and 23%, respectively (p = 0.0006). Surgery combined with RTH provides comparable results to gross tumor resection in terms of oncologic outcome in craniopharyngioma patients. Adjuvant irradiation applied in primary or salvage treatment improves disease control. The rate of complications is high irrespective of improved surgical and radiotherapeutic management.

Surgical Treatment of Pediatric Incidentally Found Brain Tumors: A Single-Center Experience.

There remains much debate about the correct management of incidentally found brain tumors in the pediatric population. This study aimed to evaluate the efficacy and safety of surgical treatment of incidentally found pediatric brain tumors. A retrospective analysis of pediatric patients who underwent surgical resection of incidentally found brain tumors between January 2010 and April 2016 was performed. A total of seven patients were included. The median age at the time of diagnosis was 9.7 years. The reasons for performing neuroimaging were as follows: impeded speech development (n = 2), shunt control (n = 1), paranasal sinuses control (n = 1), behavior changes (n = 1), head trauma (n = 1), and preterm birth (n = 1). Five patients underwent gross total tumor resection (71.4%), while subtotal resection was performed in two patients (28.6%). There was no surgery-related morbidity. Patients were followed up for a mean of 79 months. One patient with atypical neurocytoma experienced tumor recurrence 45 months following primary resection. All patients remained neurologically intact. The majority of pediatric incidentally found brain tumors were histologically benign. Surgery remains a safe therapeutic approach associated with favorable long-term outcomes. Considering the expected long lifetime of pediatric patients, as well as the psychological burden associated with having a brain tumor as a child, surgical resection can be considered an initial approach.

Predictive Value of Motor Evoked Potentials in the Resection of Intradural Extramedullary Spinal Tumors in Children.

This study aimed to evaluate the predictive value of motor evoked potentials (MEP) in the resection of pediatric intradural extramedullary (IDEM) tumors. Additionally, we aimed to assess the impact of MEP alerts on the extent of tumor resection. Medical records of pediatric patients who underwent resection of IDEM tumors with the assistance of MEP between March 2011 and October 2020 were reviewed. The occurrence of postoperative motor deficits was correlated with intraoperative MEP alerts. Sixteen patients were included. MEP alerts appeared in 2 patients (12.5%), being reflective of new postoperative motor deficits. Among the remaining 14 patients without any intraoperative MEP alerts, no motor decline was found. Accordingly, MEP significantly predicted postoperative motor deficits, reaching sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 100% (p < 0.001). In the absence of MEP alerts, 11 out of 14 patients (78.6%) underwent GTR, while no patient with intraoperative IONM alerts underwent GTR (p = 0.025). Although MEP alerts limit the extent of tumor resection, the high sensitivity and PPV of MEP underline its importance in avoiding iatrogenic motor deficits. Concurrently, high specificity and NPV ensure safer tumor excision. Therefore, MEP can reliably support surgical decisions in pediatric patients with IDEM tumors.

The features of the glymphatic system.

The glymphatic system creates a network of perivascular channels. It is made of astroglia cells, whose perikaryon extensions strongly express aquaporin-4 water channels (AQP4). The pathways of the glymphatic system ensure the transport of nutrients, including glucose, lipids, amino acids, neurotransmitters, antigens, and immune cells, as well as exchange of information via afferent and efferent immune pathways. Within the glymphatic system, convective exchange of cerebrospinal fluid (CSF) and interstitial fluid (ISF) components takes place, through aquaporin-4 water channels that facilitate fluid exchange. The proper functioning of the glymphatic system allows elimination and reabsorption of solutes, metabolites, pursuit of water and ionic balance, transport of lipid signaling molecules, regulation of intracranial pressure, cerebrospinal fluid pressure, and interstitial fluid pressure. The functions of the glymphatic system are primarily affected by the influence of the sympathetic and parasympathetic innervation, sleep and wakefulness cycle, the aging process, genetic factors, and body posture. Now, the glymphatic system shows weak activity during wakefulness, while its activity increases dramatically during sleep and the state of anesthesia. Changes occurring with age begin a number of factors that impair the function of the glymphatic system pathways. Dysfunction of the glymphatic pathways causes the aggregation of incorrectly formed proteins that underlie the development of neurodegenerative diseases. Harmful protein aggregates cause prolonged inflammation. All pathologies occurring within the central nervous system (CNS), both neurodegenerative diseases and injuries, disrupt the drainage of glymphatic pathways, which are important efflux of interstitial substances and byproducts of CNS metabolism.