Duraplasty with autologous cervical fascia in pediatric posterior fossa tumor surgery: a single-center experience with 214 cases.

PURPOSE: Posterior fossa surgeries for pediatric tumors pose challenges in achieving optimal dural repair and duraplasty is usually required. Autografts, allografts, xenografts, and synthetic substitutes can be used for duraplasty. Autologous cervical fascia can be a safe and reliable graft option for duraplasty after posterior fossa surgeries. This study aims to investigate the outcomes of duraplasty with autologous cervical fascial graft in children after posterior fossa surgery for pediatric brain tumors. METHODS: Pediatric patients with posterior fossa tumor who underwent surgery between March 2001 and August 2022 were retrospectively reviewed. Data on demographics, preoperative symptoms, diagnosis, tumor characteristics, hydrocephalus history, and postoperative complications, including cerebrospinal fluid (CSF) leakage, pseudomeningocele, and meningitis were collected. Logistic regression analysis was performed to explore risk factors for postoperative complications. RESULTS: Patient cohort included 214 patients. Autologous cervical fascia was used in all patients for duraplasty. Mean age was 7.9 ± 5.3 years. Fifty-seven patients (26.6%) had preoperative hydrocephalus and 14 patients (6.5%) received VPS or EVD perioperatively. Postoperative hydrocephalus was present in 31 patients (14.5%). Rates of CSF leak, pseudomeningocele, and meningitis were 4.2%, 2.8%, and 4.2% respectively. Logistic regression analysis revealed that postoperative EVD and VPS placement were the factors associated with postoperative complications. CONCLUSION: Autologous cervical fascia is a safe and reliable option for duraplasty with minimal risk of postoperative complications. The straightforward surgical technique and with no additional cost for harvesting the graft renders autologous cervical fascia a favorable alternative for resource-limited countries or surgical settings.

In vivo study of the utility of selective intra-arterial injection of thiopental for neuroprotection in reversible cerebral ischemia.

BACKGROUND: Neuroprotective agents are needed to reduce cerebral damage during surgical or neurointerventional procedures including stroke patients. PURPOSE: To evaluate if thiopental can be used as a neuroprotective agent when injected intra-arterially in a transient ischemia model. MATERIAL AND METHODS: In total, 24 rabbits were studied as four groups of six animals. Group 1 served as the control group. In group 2, transient ischemia was obtained by intracarotid administration of degradable starch microspheres (DSM). Group 3 was administered thiopental intra-arterially via the carotid artery. Group 4 (experimental group) received both thiopental and DSM intra-arterially. DSM and thiopental were administered through a microcatheter placed into the common carotid artery via the central ear artery access. After sacrifice, apoptotic cells in the cerebral tissues of the animals were evaluated in H&E and TUNEL stained slides. RESULTS: There was a significant increase in the number of apoptotic glial or neuronal cells in group 2 compared to the control group and group 3. The mean number of both the apoptotic neuronal cells (6.8 ± 2.1 vs. 2.5 ± 1.3, P < 0.001) and the apoptotic glial cells (9.4 ± 3.1 vs. 4.6 ± 1.6, P < 0.001) were higher in group 2 compared to group 4. In addition, a higher level of neurological improvement was observed in group 4 compared to group 2 based on neurological assessment score. CONCLUSION: The intra-arterial administration of thiopental has a protective effect on both glial and neuronal cells during temporary cerebral ischemia in low doses.

Reduced myelin in patients with isolated hippocampal sclerosis as assessed by SyMRI.

PURPOSE: Synthetic MRI (SyMRI) enables to quantify brain tissue and morphometry. We aimed to investigate the WM and myelin alterations in patients with unilateral hippocampal sclerosis (HS) with SyMRI. METHODS: Adult patients with isolated unilateral HS and age-matched control subjects (CSs) were included in this study. The SyMRI sequence QRAPMASTER in the coronal plane perpendicular to the hippocampi was obtained from the whole brain. Automatic segmentation of the whole brain was processed by SyMRI Diagnostic software (Version 11.2). Two neuroradiologists also performed quantitative analyses independently from symmetrical 14 ROIs placed in temporal and extratemporal WM, hippocampi, and amygdalae in both hemispheres. RESULTS: Sixteen patients (F/M = 6/10, mean age = 32.5 ± 11.3 years; right/left HS: 8/8) and 10 CSs (F/M = 5/5, mean age = 30.7 ± 7 years) were included. Left HS patients had significantly lower myelin and WM volumes than CSs (p < .05). Myelin was reduced significantly in the ipsilateral temporal lobe of patients than CSs, greater in left HS (p < .05). Histopathological examination including luxol fast blue stain also revealed myelin pallor in all of 6 patients who were operated. Ipsilateral temporal pole and sub-insular WM had significantly reduced myelin than the corresponding contralateral regions in patients (p < .05). No significant difference was found in WM values. GM values were significantly lower in hippocampi in patients than CSs (p < .05). CONCLUSION: SyMRI revealed myelin reduction in the ipsilateral temporal lobe and sub-insular WM of patients with HS. Whether this finding correlates with electrophysiological features and SyMRI could serve as lateralization of temporal lobe epilepsy need to be investigated.

The effect of pentoxifylline on cerebral vasospasm following experimental subarachnoid hemorrhage.

OBJECTS: Cerebral vasospasm is an important event that occurs following subarachnoid hemorage which has significant mortality and morbidity. The goal in this study was to investigate the effect of pentoxifylline on vasospasm in an experimental subarachnoid hemorrhage model. METHODS: In this study, 20 male New Zeland White rabbits weighing 3000-3500 g were assigned randomly to four groups. Animals in group 1 served as controls. Animals in group two received only intravenous pentoxifylline injection 3 times in 12 h intervals. In group 3, SAH was induced and no injection was given. Animals in group 4 received intravenous pentoxifylline (6 mg/kg) injections 3 times at 12th, 24th and 36th hours after subarachnoid hemorrhage induction. All animals were sacrificed and basilar arteries were removed at 48th hour. Basilar artery vessel diameters, wall thicknesses and luminal section areas were measured with Spot for Windows version 4.1. Statistical analysis was performed using ANOVA and Kruskall-Wallis tests. RESULTS: Mean basilar artery luminal section areas and luminal diameters in group 4 were significantly higher compared to group 3 (p < 0.05). Basilar artery wall thicknesses and were found to be higher in group 3 than in other groups and this was also statistically significant (p < 0.05). CONCLUSION: Our study demonstrated that intravenous administration of pentoxifylline significantly decreases vasospasm after subarachnoid hemorrhage.

Long-term effects of vagus nerve stimulation in refractory pediatric epilepsy: A single-center experience.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used as an adjunctive therapy for both children and adults with refractory epilepsy, over the last two decades. In this study, we aimed to evaluate the long-term effects and tolerability of VNS in the pediatric drug-resistant epilepsy (DRE) and to identify the predictive factors for responsiveness to VNS. METHODS: We retrospectively reviewed the medical records of pediatric patients who underwent VNS implantation between 1997 and 2018. Patients with ≥50% reduction of seizure frequency compared with the baseline were defined as "responders". The clinical characteristics of responders and nonresponders were compared. RESULTS: A total of 58 children (male/female: 40/18) with a mean follow-up duration of 5.7 years (3 months to 20 years) were included. The mean age at implantation was 12.4 years (4.5 to 18.5 years). Approximately half (45%) of our patients were responders, including 3 patients (5.8%) who achieved seizure freedom during follow-up. The age of seizure-onset, duration of epilepsy, age at implantation, and etiologies of epilepsy showed no significant difference between responders and nonresponders. Responders were more likely to have focal or multifocal epileptiform discharges (63%) on interictal electroencephalogram (EEG), when compared to nonresponders (36%) (p = .07). Vocal disturbances and paresthesias were the most common side effects, and in two patients, VNS was removed because of local reaction. CONCLUSION: Our series had a diverse etiological profile and patients with transition to adult care. Long-term follow-up showed that VNS is an effective and well-tolerated treatment modality for refractory childhood onset epilepsy. Age at implantation, duration of epilepsy and underlying etiology are not found to be predictors of responsiveness to VNS. Higher response rates were observed for a subset of patients with focal epileptiform discharges.

International consensus classification of hippocampal sclerosis and etiologic diversity in children with temporal lobectomy.

INTRODUCTION: The distribution of hippocampal sclerosis (HS) subtypes, according to the classification of the International League Against Epilepsy (ILAE), has been reported mainly in adult patients. We aimed to review the pathological findings in children who had anterior temporal lobectomy accompanied with amygdalohippocampectomy, in view of the current classification, and evaluate postsurgical outcome with respect to HS subtypes in childhood. METHODS: Seventy children who underwent temporal resections for treatment of medically refractory epilepsy, with a minimum follow-up of 2 years, were included; the surgical hippocampus specimens were re-evaluated under the HS ILAE classification. RESULTS: Neuropathological evaluations revealed HS type 1 in 38 patients (54.3%), HS type 2 in 2 (2.8%), HS type 3 in 21 patients (30%), and no HS in 9 patients (12.9%). Of 70 patients, 23 (32.9%) had dual pathology, and the most common pattern was HS type 3 with low-grade epilepsy-associated brain tumors (LEAT). The distribution of HS types with respect to age revealed that HS type 3 and no HS subgroups had significantly more patients younger than 12 years, compared with those of HS type 1 (90.5%, 77.8% vs 47.4%, respectively). History of febrile seizures was higher in HS type 1. Prolonged/recurrent febrile seizures were most common in patients 12 years and older, whereas LEAT was the most common etiology in patients under 12 years of age (p < 0.001). Patients with HS type 1 had longer duration of epilepsy and an older age at the time of surgery compared with patients with HS type 3 and no HS (p: 0.031, p: 0.007). At final visit, 74.3% of the patients were seizure-free. Seizure outcome showed no significant difference between pathological subtypes. CONCLUSIONS: Our study presents the distribution of HS ILAE subtypes in an exclusively pediatric series along with long-term seizure outcome. The study reveals that the leading pathological HS subgroup in children is HS type 1, similar with adult series. Hippocampal sclerosis type 2 is significantly less in children compared with adults; however, HS type 3 emerges as the second most predominant group because of dual pathology, particularly LEAT. Further studies are required regarding clinicopathological features of isolated HS in pediatric cohort. Seizure-free outcome was favorable and similar in all HS types in children. The proportion of HS types may be better defined in pediatric patients with temporal resections, as the current HS ILAE classification becomes more widely used, and may help reveal the surgical and cognitive outcome with respect to HS types.

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.

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.

OBJECTIVE: To determine audiological outcomes of children who use a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the contralateral ear. DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PARTICIPANTS: Twelve children followed with CI and contralateral auditory brainstem implant (ABI) by Hacettepe University Department of Otorhinolaryngology and Audiology in Turkey. All children were diagnosed with different inner ear malformations with cochlear nerve aplasia/hypoplasia. CI was planned in the ear with better sound detection during behavioural testing with inserted ear phones and with better CN as seen on MRI. Due to the limited auditory and speech progress with the cochlear implant, ABI was performed on the contralateral ear in all subjects. MAIN OUTCOME MEASURES: Audiological performance and auditory perception skills of children with cochlear nerve deficiency (CND) who use bimodal electrical stimulation with CI and contralateral ABI. RESULTS: Mean age of the subjects was 84.00 ± 33.94 months. Age at CI surgery and ABI surgery was 25.00 ± 10.98 months and 41.50 ± 16.14 months, respectively. However, hearing thresholds only with CI and only with ABI did not reveal significant difference, and auditory perception scores improved with bimodal stimulation. The MAIS scores were significantly improved from unilateral CI to bimodal stimulation (P = .002). Pattern perception and word recognition scores were significantly higher with the bimodal condition when compared to CI only and ABI only conditions. CONCLUSION: Children with CND showed better performance with CI and contralateral ABI combined. Depending on the audiological and radiological results, bimodal stimulation should be advised for children with CND.

Postherpetic Anti-N-methyl-D-aspartate Receptor Encephalitis after Hemispherotomy in a Patient with Intractable Startle Epilepsy.

Herpes simplex encephalitis (HSE) has been increasingly reported after neurosurgical procedures, mostly after tumor resections in patients with a prior history of HSE. Early detection and appropriate treatment are essential to prevent high mortality of the disease; however, there are diagnostic difficulties due to nonspecific prodromal symptoms. In addition, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been reported after HSE as an immunological relapse. Here, we report a case of postherpetic anti-NMDAR encephalitis following right hemispherotomy for intractable startle-induced seizures, to emphasize the importance of early diagnosis and appropriate treatment. To our knowledge, this is the first reported case of anti-NMDAR encephalitis after postoperative HSE, and the third reported case of hemispherotomy as a curative treatment for startle epilepsy.

Immunopathology in drug resistant mesial temporal lobe epilepsy with different types of hippocampal sclerosis.

PURPOSE: There is evidence that autoimmunity has a specific role in temporal lobe seizures of limbic encephalitis patients. Our aim in this study was to investigate any histopathological clues of autoimmune process in refractory temporal lobe epilepsy (TLE) patients with different pathologically proven hippocampal sclerosis (HS) types. METHODS: 22 patients who had undergone epilepsy surgery due to mesial TLE-HS were included. The sera of patients are tested for neuronal antibodies to N-methyl-D-aspartate receptors (NMDAR), leucine-rich, glioma inactivated 1 (LGI1), contactin-associated protein 2 (CASPR2), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma-aminobutyric acid B receptor (GABABR) and glutamic acid decarboxylase (GAD). Pathological and immunohistochemical investigations including neuronal nuclei (NeuN), NMDAR, GAD, glial fibrillary acidic protein (GFAP), CD8+-CD3+ lymphocytes and immunoglobulin G (IgG) were done. Patients were grouped according to type of HS. Clinical features and immunohistochemical changes were defined in these groups. RESULTS: Available sera of 15 patients did not have any neuronal antibodies. Thirteen of 22 patients had HS type 1, three had HS type 2 and two had HS type 3. According to immunohistochemical investigations CD3+ and CD8+ T cell infiltration was more prominent in the hippocampus of patients with classical HS (International League Against Epilepsy (ILAE) Type 1 HS) and there was a significant negative correlation between epilepsy duration and numbers of CD3+-CD8+ lymphocytes in temporal lobe parenchyma. CONCLUSION: The role of T cell-mediated immunopathology and immunopathological difference in a variety of drug resistant TLE-H2S patients was suggested. These findings can be helpful in understanding the epileptogenicity of HS.

Comparison of Musculocutaneous and Fasciocutaneous Free Flaps for the Reconstruction of the Extensive Composite Scalp and Cranium Defects.

Composite scalp and cranium defects, which require microsurgical reconstruction, result from tumor resection, radiation, trauma, severe burn injuries, and rarely vasculitic disorders. In the current study, the authors aim to compare the outcomes of the fasciocutaneous flaps and musculocutaneous free flaps used for the reconstruction of extensive composite scalp and cranium defects. From 2010 to 2017, 21 patients who underwent composite scalp and cranium defect reconstruction with a free flap were retrospectively identified. Eighteen patients had squamous cell carcinoma, 2 patients had meningioma, and 1 patient had Ewing sarcoma. Thirteen musculocutaneous free flaps including latissimus dorsi and vertical rectus abdominis flaps and 9 free fasciocutaneous flaps including radial forearm and anterolateral thigh flaps were used. Only 1 flap loss was encountered. No neurologic impairment in postoperative period was reported. The mean length of stay in the hospital, the duration of surgery, and total volume of blood transfusion for the fasciocutaneous flap group were significantly shorter than those for musculocutaneous flap group. No flap atrophy was reported in fasciocutaneous flap group. Reconstruction of the composite scalp and cranium defects with fasciocutaneous free flaps allows shorter hospitalization, less blood transfusion and less flap atrophy than those of musculocutaneous flaps. To this respect, their usage should be prioritized in such challenging patients.

An extremely rare complication following frontoorbital advancement: infarction of the recurrent artery of Heubner.

Many intracranial as well as extracranial complications can be seen following craniosynostosis surgeries. In this article, we would like to share an extremely rare complication of the infarction of the recurrent artery of Heubner, occurred following frontoorbital advancement. In this case, an 18-month-old male patient underwent supraorbital bar and frontal bone remodeling surgery for nonsyndromic metopic suture synostosis. The preoperative neurosurgical evaluation revealed no signs of increased intracranial pressure. On the 3rd postoperative day, the patient developed asymmetric smile and weakness on the left extremities. Computerized tomography showed a hypodense infarction region around the right basal ganglia and internal capsule, concordant with the region supplied by the recurrent artery of Heubner. The patient's symptoms started to regress on the 2nd day of enoxaparine treatment and he was discharged on 12th postoperative day with almost no signs of the event. In this paper, we presented an unlikely complication after frontoorbital advancement. Keeping in mind the long operating time and the proximity of the procedure to the central nervous system, assessment of the neurological function of the patients both before and after the operation and rapid intervention in case of development of neurologic symptoms are of great importance.

Distraction of Fronto-Orbital Segment as a Nonvascularized Bone Graft in Craniosynostotic Patients.

Fronto-orbital advancement using distraction techniques involves the dura left attached to the osteotomized bone segment to avoid dead space formation and dural injury, whereas it is impossible to reshape the supraorbital bar and the frontal bone complex, when necessary. Our approach combines advantageous parts of conventional and distraction osteogenesis techniques as remodel and distract the supraorbital bar and frontal bone complex as a free bone graft. Twenty-seven patients either being syndromic and nonsyndromic craniosynostosis, with at least 3 years of follow-up were reviewed in this study. Mean age of the patients at the time of the operation was 23.44 ±â€Š18.42 months and mean operative time was 4.96 ±â€Š0.97 hours. Blood transfusion was required in all patients, with an average of 112.04 ±â€Š44.60 mL. Amount of the distraction ranged 10 to 30 mm, a mean of 17.26 ±â€Š4.71 mm for the right side and 18.15 ±â€Š4.69 mm for the left side. Mean duration of consolidation was 98.26 ±â€Š12.98 days and mean follow-up was 41.33 ±â€Š22.92 months. In this study, result of internal distraction of fronto-orbital segment as a nonvascularized bone graft in craniosynostotic patients is reviewed to emphasize the efficacy of the nonvascularized bone graft distraction in management of craniosynostosis. Graft distraction after fronto-orbital and cranial vault remodeling appears to be safe and effective approach in correcting severe craniosynostosis deformities especially necessitating asymmetrical advancement.

De novo formation of brain tumors in pediatric population following therapeutic cranial irradiation.

PURPOSE: Tumors of central nervous system (CNS) are accounted as the second most common tumors in childhood (21% of all cancers) following hematologic malignancies. The patients can suffer more than one cancer in their lifetime, and radiotherapy is claimed for de novo formation of a new tumor in years after treatment of an initial tumor. METHODS: We have retrospectively analyzed our database for defining radiotherapy-related de novo brain tumors operated in between January, 2000 and August, 2012. New tumors in the field of radiotherapy were included into the study. RESULTS: The retrospective analysis revealed six patients (two girls and four boys) eligible for further evaluation. The children were irradiated at an age in the range of 5 and 12 years (mean, 7.8). The primary diagnoses were hematological malignancies in two (acute lymphocytic leukemia and non-Hodgkin lymphoma (NHL)) and solid tumors in other four (two pilocytic astrocytoma, one craniopharyngioma, and one grade 3 astrocytoma). All patients received cranial radiotherapy. The mean latency period for the "second tumor" was 9.5 years. The pathological diagnoses for de novo new tumors were as follows: glial tumor (two cases), medulloblastoma (two cases), mesenchymal tumor (one case), and meningeal sarcoma (one case). All de novo tumors were high graded. Mean survival was 14.6 months. CONCLUSIONS: de novo brain tumors after irradiation has poor prognosis in neurosurgical practice. Vigilance and awareness for possibility of de novo new tumor are warranted for both families and physicians at follow-ups even years after the treatment of initial tumors.

Enigmatic entity in childhood: clival chordoma from a tertiary center's perspective.

PURPOSE: Chordoma is a rare neoplasm that arises from embryonic notochordal remnants along the axial skeleton (i.e., clivus, sacrum) and the vertebral bodies. They comprise less than 1 % of CNS tumors and 1-4 % of all bone malignancies. It rarely affects children and adolescents (<5 %). Chordomas are locally aggressive and highly recurrent. Their management is challenging for clinicians. METHODS: This retrospective study includes six pediatric patients with pathological evidence of clival chordoma. These cases were identified over a period of 15 years in a tertiary care institute. RESULTS: There were two boy and four girls with a mean age of 10.6 years (range, 4-16 years). The chief complaint was due to cranial nerve palsy (or dysfunction), mostly affecting lower cranial nerves (66.6 %), followed by diplopia and headache. One patient had obstructive sleep apnea. All patients were operated and a total of 15 surgeries were performed (mean, 2.5). Tumor recurrence was observed in four patients (67 %). Two-year and 5-year progression-free survivals (PFS) were 67 and 33 %, respectively. None of the patients were lost either during the surgery or the follow-up period (6.9 years: 1-14 years). CONCLUSIONS: Clival chordomas are challenging tumors in neurosurgical practice. A multidisciplinary approach is warranted in each patient. Today, the best management strategy seems to be surgical resection followed by radiotherapy. Chemotherapy should be considered in selective and preference basis. Sharing institutional experiences will provide future insights in prognosis of these rare tumors. Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival chordomas.

Musicogenic and spontaneous seizures: EEG analyses with hippocampal depth electrodes.

Musicogenic seizure is classified as a rare form of complex reflex seizures. We present a patient with musicogenic seizures from whom invasive recordings were obtained using subdural arrays, as well as hippocampal depth electrodes. Interestingly, this patient had both spontaneous seizures and musicogenic seizures, and they originated from different hippocampi. Due to bilateral independent musicogenic seizures and spontaneous seizures, our patient was not eligible for surgery, but vagal nerve stimulation treatment was almost successful. [Published with video sequence].

Closed manual reduction maneuver of atlantoaxial rotatory dislocation in pediatric age.

PURPOSE: The purposes of this study were to characterize the clinical and radiological features of type 1 and type 2 atlantoaxial rotatory dislocations (AARD) and to evaluate the outcome of the manual reduction maneuver for these types of dislocations in pediatric patients. METHODS: This study considered 12 pediatric patients with AARD who were treated between January 2003 and March 2013. The diagnosis was established by clinical findings and 3D-CT. All of the patients were treated by closed manual reduction and then a cervical orthosis was performed. All of the patients were followed up at regular intervals. RESULTS: The causes of the AARD were trauma (91.67 %) and infection (8.33 %). The time between onset of symptoms and admission to the hospital ranged from 16 days to 6 months. In radiological evaluation, the mean rotational angulation of the patients was 30.58°. The 3D-CT examination showed that six patients (50 %) had type 1 dislocation and six patients (50 %) had type 2 dislocation. All of the patients were treated by closed manual reduction and then a cervical orthosis was performed. For 11 patients (91.67 %), dislocation was successfully reducted and maintained. One patient (8.33 %) underwent surgery, following recurrence of the second closed reduction maneuver. CONCLUSION: This preceding treatment method has successfully produced a fast and recurrence-free alignment on all except one of our patients. This technique can be a useful treatment alternative in carefully selected AARD cases. Future research is encouraged to improve decision making in the application of this treatment method and provide additional validation of the current findings.

Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases.

BACKGROUND: Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children. METHODS: We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution. RESULTS: The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p = 0.011), and more overt hemorrhages (81 vs 47 %; p = 0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete resections. Mean follow-up duration was 6.9 ± 4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up. CONCLUSIONS: Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs.

Thalamic tumors in children.

INTRODUCTION: Thalamic tumors are rare tumors which are usually diagnosed in the pediatric age group. Although recent developments in neurosurgical practice allow more radical treatments, information about outcome is scarce for these deep-seated challenging tumors. METHODS: Medical records of 45 pediatric patients who presented with thalamic tumors between 1999 and 2012 were reviewed. DISCUSSION: Prognostic implication of tumor characteristics and patient variables are discussed. Although challenging, recent innovations in the field of neurosurgery and refinements in technique may prolong survival in some cases.

Molecular Subgrouping Based on Immunohistochemistry in Medulloblastoma: a Single-Center Experience.

AIM: Medulloblastomas (MBs) are the most commonly observed malignant brain tumors affecting children; they can be classified into molecular subgroups based on the 2016 and 2021 WHO classifications, as WNT-activated, SHH-activated and TP53-wild type, SHH-activated and TP53-mutant, and non-WNT/non-SHH. However, the molecular methods to determine these subgroups are not easily accessible for routine testing as they are expensive. Here, we investigated the efficacy of immunohistochemical methods to determine molecular subgroups and prognostic predictions of MB. MATERIAL AND METHODS: ß-catenin, GAB1, YAP1, filamin A and p53 were immunohistochemically stained, and MYC and MYCN fluorescent in situ hybridization (FISH) procedures were applied to 218 cases in our series. RESULTS: Based on the histomorphological characteristics of the cases, 67.9% were deemed classic MB; 15.6% as desmoplastic/nodular medulloblastoma (DNMB); 12.8% as large cell/anaplastic (LC/A) MB; 3.7% as medulloblastoma with extensive nodularity (MBEN). Molecular characteristics revealed that 50.5% had non-WNT/non-SHH; 33.9% had SHH-activated and TP53-wildtype; 8.7% had WNT-activated; 6.9% had SHH-activated and TP53-mutant. According to the survival curves, LC/A MBs or non-WNT/non-SHH tumors showed the worst prognosis, whereas DNMBs and WNT-activated tumors showed the best prognosis. Classic MBs or SHH-activated tumors showed a moderate course. MYCN amplification was found to act as an independent poor prognostic factor in the study. CONCLUSION: The distribution of histological subtypes and molecular subgroups, amplification rates, and prognostic data obtained through immunohistochemical methods in our study were consistent with those reported in the literature. It was therefore hypothesized that the determination of molecular subgroups by immunohistochemical methods can be useful in daily diagnostic practice, especially in centers with limited access to molecular techniques.