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.

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.

Traumatic subgaleal hematoma in patient with Ehlers-Danlos syndrome: A rare case report.

Background: A subgaleal hematoma (SGH) describes scalp bleeding in the potential space between the periosteum and the galea aponeurosis. This hematoma generally occurs after vacuum-assisted and forceps delivery, but may also be seen following head trauma. Despite its benign course, SGHs may complicate by life-threatening events. Case Description: We report a case of a 10-year-old male with Ehlers-Danlos syndrome presenting with scalp swelling following minor head trauma. On examination, a small swelling was observed in the occipital region. During the follow up, as the volume of subgaleal hematoma was increasing, we performed needle aspiration to achieve volume reduction, and dressed with a cap like bandage that wrapped and compressed scalp. The patient was hospitalized due to hemodynamic instability and a blood transfusion was performed. Due to extended usage of compressive bandage, a large area of scalp tissue became necrotic. The necrotic scalp tissue was debrided and reconstructed by plastic and reconstructive surgery. After surgery, another hematoma formed extending from the front of the ear to the ipsilateral neck caused facial paralysis, this hematoma was evacuated and a drain was placed. The patient was followed up for 1 year and no recurrent cephalhematoma was observed. Conclusion: Ehlers-Danlos is a rarely encountered connective tissue syndrome, this case underscores the importance for neurosurgery physicians to recognize the potential catastrophes, these patients may present with following even minor injury.

Does Gross Total Resection Improve Progression-Free and Overall Survival in Pediatric Intracranial Ependymomas? Single-Center Clinical Experience Of 61 Cases.

OBJECTIVE: To share our clinical experience of 25 years and identify prognostic factors for progression-free and overall survival in pediatric intracranial ependymomas. METHODS: In total, 61 children who were treated between 1995 and 2020 in a single institution were included in the study. Medical records of the patients were retrospectively reviewed to obtain and analyze the following data: patient age at first surgery, sex, presenting symptoms, hydrocephalus and any invasive treatment, anatomic site, extent of resection, pathologic grade, time to progression, and time to death. Progression-free and overall survival rates and affecting factors were analyzed by Kaplan-Meier method. RESULTS: Dysphagia, number of surgeries, and spinal seeding were associated with progression free and overall survival in univariate analysis. The extent of resection, World Health Organization grade, and visual problems were also associated with progression whereas sex was associated with overall survival. Cox regression identified the extent of resection and single surgery as an independent prognostic factor for progression-free survival. No independent factor was found for overall survival. CONCLUSIONS: This single center experience of 25 years confirms the beneficial effect of gross total resection on disease progression. Although spinal seeding seems to affect survival rates, greater number of cases are needed to reveal its full effect.

Lesional resective epilepsy surgery in childhood: Comparison of two decades and long-term seizure outcome from a single center.

OBJECTIVE: Epilepsy surgery has shown efficacy in children. We aimed to assess long-term seizure outcome in children who underwent epilepsy surgery and determine predictive factors for seizure freedom. METHODS: This is a retrospective study of 196 children who underwent epilepsy surgery between 1994 and 2015 and had a minimum postoperative follow-up of 5 years. RESULTS: The median age at the time of surgery was 9.5 (0.08-19.8) years; 110 (56.1%) had temporal, 62 (31.6%) had extratemporal resections, and 24 (12.2%) had hemispheric surgery. The duration of postsurgical follow-up was between 5 and 20 years (mean±SD: 7 ± 3.2). Overall, 129 of 196 (65.8%) patients had Engel class I outcome at final visit. Among patients who underwent temporal, extratemporal and hemispheric surgery; 84 of 110 (76.4%), 34 of 62 (54.8%), and 11 of 24 (45.8%) patients had complete seizure freedom, respectively (p: 0.016). Patients with tumors had the best outcome, with 83.1% seizure freedom. The number of preoperative antiseizure medications (OR 3.19, 95% CI 1.07-9.48), the absence of postoperative focal epileptiform discharges (OR 8.98, 95% CI 4.07-19.79) were independent predictors of seizure freedom. Across two decades, the age at surgery was decreased (p: 0.003), overall seizure freedom (61.8% vs 68%) did not differ. In the past decade, a higher proportion of malformations of cortical development was operated (14.7% vs 35.9%, p: 0.007). SIGNIFICANCE: Our findings showed favorable long-term seizure outcome in children who underwent epilepsy surgery. The results are encouraging for developing centers with limited resources to establish pediatric epilepsy programs.

Challenges in the Intraoperative Consultation of Low-Grade Epilepsy-Associated Neuroepithelial Tumors by Cytomorphology in Squash Preparations.

INTRODUCTION: Low-grade epilepsy-associated neuroepithelial tumors (LEATs) create a diagnostic challenge in daily practice and intraoperative pathological consultation (IC) in particular. Squash smears are extremely useful in IC for accurate diagnosis; however, the knowledge on cytopathologic features of LEATs is based on individual case reports. Here, we discuss the 3 most common and well-established entities of LEATs: ganglioglioma (GG), dysembryoplastic neuroepithelial tumor (DNT), and papillary glioneuronal tumor (PGNT). METHODS: Thirty patients who underwent surgery for GG, DNT, and PGNT between 2001 and 2021 were collected. Squash smears prepared during intraoperative consultation were reviewed by 1 cytopathologist and an experienced neuropathologist. RESULTS: Among the 30 tumors, 16 (53.3%) were GG, 11 (36.6%) DNT, and 3 (10%) PGNT. Cytomorphologically, all of the 3 tumor types share 2 common features such as dual cell population and vasculocentric pattern. GG smears were characteristically composed of dysplastic ganglion cells and piloid-like astrocytes on a complex architectural background of thin- to thick-walled vessels. DNT, on the other hand, showed oligodendroglial-like cells in a myxoid thin fibrillary background associated with a delicate capillary network. Common cytological features of PGNT were hyperchromatic cells with narrow cytoplasm surrounding hyalinized vessels forming a pseudopapillary pattern and bland cells with neuroendocrine nuclei dispersed in a neuropil background. CONCLUSION: A higher diagnostic accuracy can be obtained when squash smears are applied with frozen sections. However, it is important to integrate clinical and radiologic features of the patient as well as to know the cytopathologic features of the LEAT spectrum in the context of differential diagnosis to prevent misinterpretation in the IC.

Pediatric Neuroblastoma with Brain Metastasis: The Prognostic Role of Surgery.

AIM: To analyze the prognostic factors and treatment outcomes of neuroblastoma patients with brain metastasis. MATERIAL AND METHODS: The medical records of 7 patients with brain metastasis among 120 neuroblastoma patients treated with radiotherapy between June 2002 and December 2018 at our department were collected retrospectively. Survival time and prognostic factors were evaluated. RESULTS: The prevalence of brain metastasis was 7/120 (5.8%). The median age was 35 months (25-64). Three (43%) patients had single brain metastasis. Two (29%) patients with solitary metastases underwent gross total resection and 1 (14%) patient with two lesions underwent subtotal resection for brain metastasis. All patients received chemotherapy and radiotherapy. The median time interval between the initial diagnosis and the brain metastasis was 14 months (0-28 months) in all patients; 12 months (0-28 months) in deceased patients and 23 months (19-26 months) in patients who were still alive at the time of this analysis (p=0.245). The median overall survival time after the treatment of brain metastasis was 14 months (7-22 months). Five patients died from progressive disease and 2 patients who had gross total resection were alive for 164 and 187 months, respectively. While the 5-year overall survival rate was 67% in patients with isolated brain metastasis, the 5-year overall survival rate was 0% in the presence of other metastases (p=0.221). CONCLUSION: Isolated brain metastasis and gross total resection of the brain metastasis seems to be favorable prognostic factors. Patients with solitary brain metastasis should be consulted for neurological surgery.

Revision Surgery for Pediatric Nontumor Auditory Brainstem Implant Users: Audiological, Rehabilitative, and Surgical Outcomes.

OBJECTIVE: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. PATIENTS: Five pediatric ABI users who had revision surgery for device malfunctions. INTERVENTIONS: Revision surgery for ABI malfunctions. MAIN OUTCOME MEASURES: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared. RESULTS: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed. CONCLUSION: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development.

Temporal encephaloceles can be missed in patients with refractory temporal lobe epilepsy.

Temporal encephaloceles (TEs) are one of the cause of refractory temporal lobe epilepsy (TLE). We reviewed the neuroimaging and video-electroencephalography (EEG) records of epilepsy patients who underwent temporal lobectomy in our center to investigate frequency of TEs. We retrospectively reevaluated 294 patients who underwent epilepsy surgery in our tertiary epilepsy centre between January 2010 and March 2019 and included 159 patients (78 females, 49 %; 81 males) who had temporal lobectomy. Preoperatively, TEs were reported in 3 of 159 patients (1 female, 2 males). After reevaluation 4 more patients with TEs (1 female, 3 males) were added. The ratio of TE in patients who underwent temporal lobectomy increased from 1.8 % (n=3) to 4,4 % (n=7). The median ages were 18 (range 16-22) versus 10 years (range 5-17) at habitual seizure onset and the median of epilepsy duration was 5 (range 3-15) versus 175 (range 11-25) years between patients with and without TE. Habitual seizure onset age was significantly higher (p =, 007) in the patients with encephalocele and epilepsy duration was shorter (p =, 003) than patients without encephalocele. The ictal EEG records of all patients TE rhythmic delta activity which is suggested neocortical temporal lobe onset seizures. 4 of 7 patients' PET imaging showed temporal lobe hypometabolism compatible with ipsilateral to the TEs. The three patients underwent anterior temporal lobectomy without amygdalohippocampectomy and others had anterior temporal lobectomy with amygdalohippocampectomy. We suggested that there might be some clues for temporal encephalocele, an easily overlooked cause in patients with nonlesional temporal lobe epilepsy.TLE patients with TE had relatively late onset of epilepsy and rhythmic delta activity on ictal EEG. Also, temporal hypometabolism on PET may be a useful key to suspicion of TE.

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.

Effects of Age at Auditory Brainstem Implantation: Impact on Auditory Perception, Language Development, Speech Intelligibility.

OBJECTIVE: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility. STUDY DESIGN: Retrospective single cohort design. SETTING: Tertiary referral center. PATIENTS: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ±â€Š6.5) months in the early group, 45.53 (ranged ±â€Š7.9) months in the late group. INTERVENTION(S): Retrosigmoid craniotomy and ABI placement. MAIN OUTCOME MEASURE(S): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room. RESULTS: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps. CONCLUSIONS: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

Long-Term Outcomes of Pediatric Meningioma Surgery: Single Center Experience with 23 Patients.

AIM: To evaluate the long-term outcomes and prognostic variables in a surgical cohort of pediatric meningiomas treated in a single institution. MATERIAL AND METHODS: Medical records and follow-up notes of 23 pediatric patients aged < 18 years (12 male and 11 female; mean age on presentation, 13.1 ± 4.4 years) harboring 27 meningiomas operated between 1994 and 01/2019 at Hacettepe University Ihsan Dogramaci Children’s Hospital were evaluated. RESULTS: One patient had neurofibromatosis (NF) type 1, and five patients had NF2. Tumors were most commonly located in the convexity (n=6) and parasagittal or falcine (n=6). Gross total resection was performed in 70.4% of cases. WHO grade I tumors accounted for 56% of all cases, whereas high-grade meningiomas accounted for 44% (33% grade II, 11% grade III). The mean follow-up duration was 10.3 ± 7.7 years. Three patients (13%) died during follow-up, and 76.2% of the patients had favorable outcome (Glasgow Outcome Scale > 3) during the last follow-up assessment. Ten patients (43.5%) had relapse. In univariate analysis, low histological grade (p=0.030) and gross total resection (p=0.024) were associated with favorable outcome. The 10-year overall survival rate was 86%. CONCLUSION: Meningiomas in the pediatric age group are surgically treatable tumors with fairly good outcomes. However, relapses are common even for low-grade tumors; therefore, long-term surveillance and aggressive treatment are needed.

Pharmacoresistant seizures in neurofibromatosis type 1 related to hippocampal sclerosis: Three case presentation and review.

Neurofibromatosis type 1 (NF1) is an autosomal dominantly inherited disorder, with an estimated prevalence of 1 in 3000-4000 people. Seizures occur 4-7% of individuals with NF1, mostly due to associated brain tumors or cortical malformations. Hippocampal sclerosis (HS) in the patients with NF1 has been reported very rarely and only 15 patients were found in review of English literature. We presented here 3 additional patients with NF1 and intractable seizures due to hippocampal sclerosis; in whom one of them underwent epilepsy surgery and he is seizure free for 5 years after right temporal lobectomy.

Survival Benefit with Gross Total Resection and Adjuvant Radiotherapy in Childhood Atypical Teratoid/Rhabdoid Tumors: Results of a Single-Center Cohort of 27 Cases.

AIM: To share a single center experience with 27 atypical teratoid/rhabdoid tumor (AT/RT) cases, and to determine the effect of gross total tumor resection and other clinical characteristics on the overall survival rate of AT/RT. MATERIAL AND METHODS: We included 27 patients-with a histopathologically confirmed primary intracranial childhood AT/ RT-who were operated in our clinic between January 2000 and December 2017. Age, sex, tumor location, disseminated disease, the presence of hydrocephalus, symptom duration till diagnosis, the extent of resection, and adjuvant radiotherapy were evaluated for their influence on overall survival. RESULTS: Median age at diagnosis for 27 patients was 19.1 months (7.2 months-5 years). Gross total resection was possible in 13 (48.72%) patients. Except for three patients who died of perioperative complications, all patients received chemotherapy and 11 received radiotherapy. In univariate analysis, male sex, older age at diagnosis (≥24 months), gross total resection, and radiotherapy were associated with overall longer survival; however, radiotherapy remained the only significant parameter in multivariate analysis. CONCLUSION: AT/RT is a rare and dreadful brain tumor that has low survival rates despite contemporary treatment. Radiotherapy seems to prolong survival; however, large-scale studies are needed to establish prognostic factors.

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.

Evaluation of spinal involvement in children with mucopolysaccharidosis VI: the role of MRI.

OBJECTIVE: To evaluate spinal MRI features of mucopolysaccharidosis (MPS) VI and to assess the correlation with clinical findings. METHODS: We retrospectively evaluated spinal MRI scans and clinical findings at the time of imaging in 14 patients (8 male, 6 female) with MPS VI. Craniometric measurements were performed and the images were assessed for bony anomalies, spinal stenosis and spinal cord compression. The degree of cervical cord compression was scored and correlated with neurological examination findings at the time of imaging. Vertebral alignment, structural changes in spinal ligaments and intervertebral discs were also assessed. RESULTS: All patients had cervical stenosis due to bony stenosis and thickened retrodental tissue (median: 6.05 mm, range 3.3-8 mm). Retrodental tissue thickness was found to increase with age (p = 0.042). Compressive myelopathy was detected at upper cervical level in 11 (79%) and lower thoracic level in 2 patients (14%). Significant inverse correlation was found between cervical myelopathy scores and neurological strength scores. The most common bony changes were hypo/dysplastic odontoid; cervical platyspondyly with anterior inferior beaking; thoracic posterior end plate depressions and lumbar posterior scalloping. Kyphosis due to retrolisthesis of the beaked lumbar vertebrae and acute sacrococcygeal angulations were other remarkable findings. CONCLUSION: MRI is an essential component in evaluation of spinal involvement in MPS VI, and scanning of the entire spine is recommended to rule out thoracic cord compression. Advances in knowledge: This study provides a detailed description of spinal MRI findings in MPS VI and underlines the role of MRI in management of cord compression.

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.