Doing more with less: surgical results of pediatric posterior fossa tumors from a single center in Latin America.

OBJECTIVE: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series. METHODS: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019. RESULTS: A total of 135 pediatric patients, with an average age of 7.5 years at diagnosis and a mean follow-up of 35.7 months, were included in the study. Most tumors were located within the midline, with ventriculomegaly observed in 71.4% of the patients. Pilocytic astrocytomas encompassed the majority of tumors (34.1%), followed by medulloblastomas (27.4%) and ependymomas (11.8%). Gross total resection (GTR) was achieved in 71.8% of the patients, with a recurrence rate of 20%. Surgical complications were observed in 25.9% of the patients. GTR significantly impacted 5-year overall survival (OS) and 4-year progression-free survival (PFS) in patients with posterior fossa tumors. Patients who underwent GTR had a 5-year OS of 89.7%, compared to 72.7% for near-total resection and 70.8% for subtotal resection. The 4-year PFS for patients who underwent GTR was 82.5%, whereas it was 63.6% for patients who underwent near-total resection and 54.2% for patients who underwent subtotal resection. CONCLUSION: Surgical resection remains the main treatment for pediatric posterior fossa tumors, and higher resection rates are linked to better survival outcomes. Despite limited resources for molecular diagnosis, our institution has demonstrated that a specialized neurooncological center with a high surgical volume can still achieve favorable survival outcomes for these patients.

Junctional instability in neuroepithelium and network hyperexcitability in a focal cortical dysplasia human model.

Focal cortical dysplasia is a highly epileptogenic cortical malformation with few treatment options. Here, we generated human cortical organoids from patients with focal cortical dysplasia type II. Using this human model, we mimicked some focal cortical dysplasia hallmarks, such as impaired cell proliferation, the presence of dysmorphic neurons and balloon cells, and neuronal network hyperexcitability. Furthermore, we observed alterations in the adherens junctions zonula occludens-1 and partitioning defective 3, reduced polarization of the actin cytoskeleton, and fewer synaptic puncta. Focal cortical dysplasia cortical organoids showed downregulation of the small GTPase RHOA, a finding that was confirmed in brain tissue resected from these patients. Functionally, both spontaneous and optogenetically-evoked electrical activity revealed hyperexcitability and enhanced network connectivity in focal cortical dysplasia organoids. Taken together, our findings suggest a ventricular zone instability in tissue cohesion of neuroepithelial cells, leading to a maturational arrest of progenitors or newborn neurons, which may predispose to cellular and functional immaturity and compromise the formation of neural networks in focal cortical dysplasia.

Interferon-beta induces major histocompatibility complex of class I (MHC-I) expression and a proinflammatory phenotype in cultivated human astrocytes.

Major histocompatibility complex class I (MHC-I) has been implicated in several types of neuroplasticity phenomena. Interferon beta-1b (IFN-ß) increases MHC-I expression by motoneurons after sciatic nerve crush in mice, improving axonal growth and functional recovery. Additionally, IFN-ß induces glial hypertrophy associated with upregulation of glial fibrillary acidic protein (GFAP) and MHC-I in murine astrocytes in vitro. As knowledge about MHC-I and its role in synaptic plasticity in human astrocytes (HAs) is scarce, we investigated these aspects in mature HAs obtained from the neocortex of patients undergoing surgery due to hippocampal sclerosis. Cells were exposed to media in the absence (0 IU/ml) or presence of IFN-ß for 5 days (500 IU/ml). Beta-2 microglobulin (ß2m), a component of the MHC-I, GFAP and vimentin proteins, was quantified by flow cytometry (FC) and increased by 100%, 60% and 46%, respectively, after IFN-ß exposure. We also performed qRT-PCR gene expression analyses for ß2m, GFAP, vimentin, and pro- and anti-inflammatory cytokines. Our data showed that IFN-ß-treated astrocytes displayed ß2m and GFAP gene upregulation. Additionally, they presented a proinflammatory profile with increase in the IL-6 and IL-1ß genes and a tendency to upregulate TNF-α. Moreover, we evaluated the effect of HAs conditioned medium (CM) on the formation/maintenance of neurites/synapses by the PC12 lineage. Synaptophysin protein expression was quantified by FC. The CM of IFN-ß-activated astrocytes was not harmful to PC12 neurites, and there was no change in synaptophysin protein expression. Therefore, IFN-ß activated HAs by increasing GFAP, vimentin and MHC-I protein expression. Like MHC-I modulation and astrocyte activation may be protective after peripheral nerve damage and in some neurodegenerative conditions, this study opens perspectives on the pathophysiological roles of astroglial MHC-I in the human CNS.

Transsylvian amygdalohippocampectomy for mesial temporal lobe epilepsy: Comparison of three different approaches.

OBJECTIVE: This study's objective was to compare the transinsular (TI-AH), transuncus (TU-AH), and temporopolar (TP-AH) amygdalohippocampectomy approaches regarding seizure control, temporal stem (TS) damage, and neurocognitive decline. METHODS: We included 114 consecutive patients with unilateral hippocampal sclerosis (HS) who underwent TI-AH, TU-AH, or TP-AH between 2002 and 2017. We evaluated seizure control using Engel classification. We used diffusion tensor imaging and postoperative Humphrey perimetry to assess the damage of the TS. We also performed pre- and postoperative memory performance and intelligence quotient (IQ). RESULTS: There were no significant differences in the proportion of patients free of disabling seizures (Engel IA+IB) among the three surgical approaches in the survival analysis. However, more patients were free of disabling seizures (Engel IA+IB) at 2 years of postsurgical follow-up with TP-AH (69.5%) and TI-AH (76.7%) as compared to the TU-AH (43.5%) approach (p = .03). The number of fibers of the inferior fronto-occipital fasciculus postoperatively was reduced in the TI-AH group compared with the TU-AH and TP-AH groups (p = .001). The rate of visual field defects was significantly higher with TI-AH (14/19, 74%) in comparison to the TU-AH (5/15, 33%) and TP-AH (13/40, 32.5%) approaches (p = .008). Finally, there was a significant postoperative decline in verbal memory in left-sided surgeries (p = .019) and delayed recall for both sides (p < .001) regardless of the surgical approach. However, TP-AH was the only group that showed a significant improvement in visual memory (p < .001) and IQ (p < .001) for both right- and left-sided surgeries. SIGNIFICANCE: The TP-AH group had better short-term seizure control than TU-AH, a lower rate of visual field defects than TI-AH, and improved visual memory and IQ compared to the other groups. Our findings suggest that TP-AH is a better surgical approach for temporal lobe epilepsy with HS than TI-AH and TU-AH.

Magnetic resonance imaging findings and clinical characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy in a predominantly adult cohort.

OBJECTIVE: We aimed to better characterize the magnetic resonance imaging (MRI) findings of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE), a rare clinicopathological entity associated with pharmacoresistance recently described in patients with frontal lobe epilepsy. METHODS: We studied 12 patients who underwent epilepsy surgery and whose surgical specimens showed histopathological findings of MOGHE, characterized by preserved cortical lamination, blurred gray-white matter interface due to increased number of oligodendrocytes, and heterotopic neurons in the white matter. The age at MRI evaluation ranged from 11 to 58 years, except for one 4.5-year-old patient. RESULTS: Following a detailed MRI analysis using an in-house protocol, we found abnormalities in all cases. The lesion was circumscribed in the frontal lobe in six (50%) and in the temporal lobe in three (25%) patients. In the remaining three patients (25%), the lesion was multilobar (frontotemporal and temporoparieto-occipital). Cortical thickening was mild in all patients, except in the 4.5-year-old patient, who had pronounced cortical thickening and white matter blurring. We also identified cortical/subcortical hyperintense T2/fluid-attenuated inversion recovery signal associated with gray/white matter blurring in all but one patient. When present, cleft cortical dimple, and deep sulci aided in localizing the lesion. Overall, the MRI findings were like those in focal cortical dysplasia (FCD) Type IIa. Surgical outcome was excellent in five patients (Engel Class I in 25% and II in 17%). The remaining seven patients (58%) had worthwhile seizure reduction (Engle Class III). Incomplete lesion resection was significantly associated with worse outcomes. SIGNIFICANCE: MRI findings associated with MOGHE are similar to those described in FCD Type IIa. Although more frequent in the frontal lobe, MOGHE also occurred in the temporal lobe or involved multiple lobes. Multilobar or extensive MOGHE MRI lesions are associated with less favorable surgical outcomes. Because this is a rare condition, multicenter studies are necessary to characterize MOGHE further.

Temporal lobe structural evaluation after transsylvian selective amygdalohippocampectomy.

OBJECTIVE: Mesial temporal lobe epilepsy (MTLE) is the most common type of focal epilepsy in adolescents and adults, and in 65% of cases, it is related to hippocampal sclerosis (HS). Selective surgical approaches to the treatment of MTLE have as their main goal resection of the amygdala and hippocampus with minimal damage to the neocortex, temporal stem, and optic radiations (ORs). The object of this study was to evaluate late postoperative imaging findings on the temporal lobe from a structural point of view. METHODS: The authors conducted a retrospective evaluation of all patients with refractory MTLE who had undergone transsylvian selective amygdalohippocampectomy (SAH) in the period from 2002 to 2015. A surgical group was compared to a control group (i.e., adults with refractory MTLE with an indication for surgical treatment of epilepsy but who did not undergo the surgical procedure). The inferior frontooccipital fasciculus (IFOF), uncinate fasciculus (UF), and ORs were evaluated on diffusion tensor imaging analysis. The temporal pole neocortex was evaluated using T2 relaxometry. RESULTS: For the IFOF and UF, there was a decrease in anisotropy, voxels, and fibers in the surgical group compared with those in the control group (p < 0.001). An increase in relaxometry time in the surgical group compared to that in the control group (p < 0.001) was documented, suggesting gliosis and neuronal loss in the temporal pole. CONCLUSIONS: SAH techniques do not seem to totally preserve the temporal stem or even spare the neocortex of the temporal pole. Therefore, although the transsylvian approaches have been considered to be anatomically selective, there is evidence that the temporal pole neocortex suffers structural damage and potentially functional damage with these approaches.

Associations of VEGFA and KDR single-nucleotide polymorphisms and increased risk and aggressiveness of high-grade gliomas.

Angiogenesis, induced by the vascular endothelial growth factor A through its ligation to the vascular endothelial growth receptor 2, has been described as a crucial point in high-grade glioma development. The aim of this study was to evaluate the influence of VEGFA-2578C/A, -2489C/T, -1154G/A, -634G/C, and -460C/T, and KDR-604T/C, -271G/A, +1192G/A, and +1719A/T single-nucleotide polymorphisms on risk and clinicopathological aspects of high-grade glioma. This case-control study enrolled 205 high-grade glioma patients and 205 controls. Individuals with VEGFA-2578 CC or CA, VEGFA-1154 GG, VEGFA-634 GC or CC, and VEGFA-460 CT or TT genotypes were under 2.56, 1.53, 1.54, and 1.84 increased risks of high-grade glioma, compared to others, respectively. And 1.61, 2.66, 2.52, 2.53, and 2.02 increased risks of high-grade glioma were seen in individuals with VEGFA-2578 CC plus VEGFA-1154 GG, VEGFA-2578 CC or CA plus VEGFA-634 GC or CC, VEGFA-2578 CC or CA plus VEGFA-460 CT or TT, VEGFA-1154 GG or GA plus VEGFA-634 GC or CC, and VEGFA 634 GC or CC plus VEGFA-460 CT or TT combined genotypes, respectively, when compared to others. The "CAGT" haplotype of KDR single-nucleotide polymorphisms was more common in patients with grade IV than in those with grade III tumors, and individuals carrying this haplotype were at 1.76 increased risk of developing grade IV tumors than others. We present, for the first time, preliminary evidence that VEGFA-2578C/A and VEGFA-1154G/A single-nucleotide polymorphisms increases high-grade glioma risk, and "CAGT" haplotype of the KDR gene alters high-grade glioma aggressiveness and risk of grade IV tumors in Brazil.

ATP Synthase Subunit Beta Immunostaining is Reduced in the Sclerotic Hippocampus of Epilepsy Patients.

Epilepsy is a common disease presenting with recurrent seizures. Hippocampal sclerosis (HS) is the commonest histopathological alteration in patients with temporal lobe epilepsy (TLE) undergoing surgery. HS physiopathogenesis is debatable. We have recently studied, by using mass spectrometry-based proteomics, an experimental model of TLE induced by electrical stimulation. Specifically, protein expressions of both the beta subunit of mitochondrial ATP synthase (ATP5B) and of membrane ATPases were found to be reduced. Here, we investigated tissue distribution of ATP5B and sodium/potassium-transporting ATPase subunit alpha-3 (NKAα3), a protein associated with neuromuscular excitability disorders, in human hippocampi resected "en bloc" for HS treatment (n = 15). We used immunohistochemistry and the stained area was digitally evaluated (increase in binary contrast of microscopic fields) in the hippocampal sectors (CA1-CA4) and dentate gyrus. All HS samples were classified as Type 1, according to the International League Against Epilepsy (ILAE) 2013 Classification (predominant cell loss in CA1 and CA4). ATP5B was significantly decreased in all sectors and dentate gyrus of HS patients compared with individuals submitted to necropsy and without history of neurological alterations (n = 10). NKAα3 expression showed no difference. Moreover, we identified a negative correlation between frequency of pre-operative seizures and number of neurons in CA1. In conclusion, our data showed similarity between changes in protein expression in a model of TLE and individuals with HS. ATP5B reduction would be at least in part due to neuronal loss. Future investigations on ATP5B activity could provide insights into the process of such cell loss.

Fractional anisotropy of the optic radiations correlates with the visual field after epilepsy surgery.

PURPOSE: This study assessed whether optic radiations (OR) microstructure after temporal lobe epilepsy (TLE) surgery correlated with visual field defects (VFD). METHODS: Patients were subjected to diffusion tensor imaging (DTI) tractography of the OR and Humphrey perimetry after TLE surgery. We used Spearman's test to verify correlations between tractographic parameters and perimetry mean deviation. Tractographic variables were compared between patients with VFD or intact perimetry. Multiple logistic regression was applied between DTI and perimetry values. DTI sensitivity and specificity were assessed with a receiver operating characteristic (ROC) curve to evaluate VFD. RESULTS: Thirty-nine patients had reliable perimetry and OR tractography. There was a significant correlation between (1) fractional anisotropy (FA) and both total (rho = 0.569, p = 0.0002) and quadrant (rho = 0.453, p = 0.0037) mean deviation and (2) radial diffusivity and total mean deviation (rho = - 0.350, p = 0.0286). There was no other significant correlation. Patients with VFD showed a significantly lower FA compared with patients with normal perimetry (p = 0.0055), and a 0.01 reduction in FA was associated with a 44% increase in presenting VFD after surgery (confidence interval, CI = 1.10-1.88; p = 0.0082). Using a FA of 0.457, DTI tractography showed a specificity of 95.2% and a sensitivity of 50% to detect VFD after surgery (area under the curve = 0.7619, CI = 0.6020-0.9218). CONCLUSION: The postoperative OR microstructure correlated with visual loss after epilepsy surgery. DTI postoperative OR tractography may be helpful in evaluating VFD.

Factors affecting diagnosis of primary pediatric central nervous system neoplasias in a developing country.

PURPOSE: Understand the variables that could interfere with diagnosis and prompt treatment in CNS childhood cancer in Brazil, a developing country with continental dimensions. METHODS: From 2005 to 2010, we retrospectively evaluated factors, which could represent a negative influence on the time period elapsing from the onset of symptoms until the diagnosis of the central nervous system (CNS) neoplasia in children and adolescents attended in our service. RESULTS: Two hundred seventeen records were analyzed retrospectively. Factors of the households were evaluated, and this data was related to the time period elapsing from presentation of the first symptoms until the diagnosis of CNS neoplasia. The average time elapsed from the onset of the symptoms until seeking medical assistance was 96 days, and from medical assistance to patient referral to a reference service was 33 days. The symptoms which most contributed to a shorter delay in diagnosis were changes in gait and paresis, mother's occupation, father's education level, patient gender, and living in the state of São Paulo. Besides that, variables such as male gender, mother's education level, and lower patient age were associated with an early diagnosis time. CONCLUSION: There is great difficulty in performing early diagnosis of CNS tumors, partly due to parent's inability to recognize signs and symptoms, and in part due to an educational deficit among healthcare professionals. Identification of measures that can minimize these causes of delay is fundamental to increasing the chance of cure and survival of these patients.

Using the keystone design perforator island flap in large myelomeningocele closure.

OBJECTIVE: Many repair techniques have been proposed to treat large myelomeningocele (MMC), and although effective in many cases, some of these techniques can be complex and time consuming, with complications such as cerebrospinal fluid (CSF) leakage, flap loss, tip necrosis, and wound dehiscence. The purpose of this study was to analyze cases of large skin defects and the methods applied and to report the outcomes of the keystone design perforator island flap (KDPIF) technique for large MMC closure. METHODS: The authors performed a retrospective review of all neonatal patients who had undergone KDPIF for MMC closure in the period from 2013 to 2018. All patients had a diagnosis of lumbosacral MMC based on obstetric ultrasound. The neurosurgeons and plastic surgeons had selected the cases after concluding that primary closure would be unlikely. The design of the flap is based on the randomly located vascular perforators, creating two identical opposing flaps to fashion a double keystone flap. During wound closure, V-Y advancement of each end of the double flap in the longitudinal axis creates redundancy in the central portion of the flap and reduces the horizontal tension. After discharge, both the neurosurgery and plastic surgery teams followed up all patients, tracking the results with photography. RESULTS: No skin flap dehiscence or necrosis, infection, or CSF leakage was detected, proving the reliability of the flap. One of the patients required further surgery for the large skin defects after insufficient intrauterine closure of the MMC and successfully underwent KDPIF treatment. Another patient (14.3%) had severe neonatal sepsis, which ultimately led to death. A ventriculoperitoneal shunt was required after the skin defect repair in 5 (83.3%) of the 6 surviving patients. Exceptional aesthetic results were achieved for all patients during the follow-up. CONCLUSIONS: The KDPIF technique is based on well-known vascular perforators of the intercostal, lumbar, and gluteal regions. Wound tension is widely distributed by the flap and, as a consequence, relevant tissue bulk, reliable vascularity, and important geometrical versatility are provided. In addition, most of the muscles and fascia are preserved, which is another advantage in terms of minimizing secondary morbidity to local tissue rearrangement. The use of KDPIF closure was successfully shown to be a viable alternative for more complex MMCs that present with large skin defects.

Is inpatient ictal video-electroencephalographic monitoring mandatory in mesial temporal lobe epilepsy with unilateral hippocampal sclerosis? A prospective study.

OBJECTIVE: To compare surgical outcome in mesial temporal lobe epilepsy (MTLE) patients with unilateral hippocampal sclerosis (MTLE-HS) who had or did not have preoperative video-electroencephalographic monitoring (VEEG). METHODS: A prospective study was undertaken with 166 consecutive pharmacoresistant unilateral MTLE-HS patients. All patients were investigated with detailed seizure semiology, serial routine outpatient EEG, magnetic resonance imaging, neuropsychological evaluation, and if necessary, other examinations. Postoperative follow-up ranged between 2 and 16 years. Patients were divided into: (1) patients operated on based on routine outpatient EEG information, with >80% of EEGs with unilateral interictal epileptiform discharges (IEDs) ipsilateral to HS or ictal events (n = 71); and (2) patients submitted to preoperative VEEG (n = 95). To avoid the bias generated by ictal recordings, we performed a subanalysis of: (1) patients without preoperatively ictal recordings (n = 80) and (2) patients with ictal recordings in VEEG or routine outpatient EEG (n = 86). RESULTS: Groups were similar regarding gender, age at surgery, seizure onset, preoperative seizure frequency, and duration of follow-up. Overall, 136/166 (81.92%) were classified as Engel I seizure outcome, with no difference between groups; 76.84% and 88.73% of patients with and without VEEG, respectively, had Engel I postoperative seizure outcome (P = .77). The time lag until surgery was shorter in the group without VEEG (80 vs 38 months; P = .01). Considering ictal recordings, 76.74% of patients with seizures recorded and 87.50% without ictal recordings had Engel I outcome (P = .11). SIGNIFICANCE: We performed the first prospective study in a tertiary epilepsy center comparing surgical outcomes in unilateral MTLE-HS patients investigated preoperatively with and without VEEG. Based on the surgical outcome, VEEG is not imperative in patients with unilateral MTLE-HS who have compatible semiology and clearly ipsilateralized IEDs evaluated by a multidisciplinary and experienced epilepsy group.

Controversies in the surgical management of congenital craniocervical junction disorders - A critical review.

INTRODUCTION: Congenital disorders of the craniovertebral junction (CVJ) include a wide range of conditions, such as Chiari malformation (CM), basilar Invagination (BI), and atlantoaxial dislocation (AAD). The objective of this paper is to critically review the literature related to the management of congenital CVJ disorders focusing on: the significant developments in the past (from anterior open ventral decompression to modern CVJ realignment); the diagnosis of CVJ instability; the role of atlantoaxial fixation without posterior fossa decompression in patients with tonsillar herniation and no evidence of CVJ instability; use of C1-2 inter-articular spacers and use of C1-2 interarticular spacers with intra-operative manipulations to correct BI with AAD along with its deformity. MATERIALS AND METHODS: We performed a review of articles showing up on PubMed database without time restriction. Articles were included according to the purpose of our review and selected by two authors (AFJ and PSC). RESULTS: CVJ instability may be inferred when there is atlantoaxial abnormal motion seen on dynamic studies, facet joint subluxation or severe symptomatic CVJ kyphosis; routine fixation of patients with CM without clear CCJ instability, while based on an interesting hypothesis, still requires further studies; use of C1-2 inter-articular spacers for re-establishing CVJ alignment is probably the most effective surgery for posterior CVJ realignment and reduction of AAD and BI, potentially avoiding the need for an open or endoscopic anterior odontoidectomy (AO); current development of deformity correcting surgeries and the measurement of joint indices to plan surgery have provided new strategies for treatment. CONCLUSION: We present a critical review of important new concepts involved in the surgical treatment of CVJ congenital disease.

Radiological evaluation of cervical spine involvement in rheumatoid arthritis.

Cervical spine involvement commonly occurs in patients with rheumatoid arthritis (RA), especially those with inadequate treatment or severe disease forms. The most common site affected by RA is the atlantoaxial joint, potentially resulting in atlantoaxial instability, with cervical pain and neurological deficits. The second most common site of involvement is the subaxial cervical spine, often with subluxation, resulting in nerve root or spinal cord compression. In this paper, the authors review the most commonly used plain radiographic criteria to diagnose cervical instabilities seen with RA. Finally, we discuss the advantages and disadvantages of cervical CT and MRI in the setting of cervical involvement in RA.

Computed tomography evaluation of the normal craniocervical junction craniometry in 100 asymptomatic patients.

OBJECT: Most of the craniometric relationships of the normal craniocervical junction (CCJ), especially those related to angular craniometry, are still poorly studied and based on measurements taken from simple plain radiographs. In this study, the authors performed a craniometric evaluation of the CCJ in a population without known CCJ anomalies. The purpose of the study was to evaluate the normal CCJ craniometry based on measurements obtained from CT scans. METHOD: The authors analyzed 100 consecutive CCJ CT scans obtained in adult patients who were admitted at their tertiary hospital for treatment of non-CCJ conditions between 2010 and 2012. A total of 17 craniometrical measurements were performed, including the relation of the odontoid with the cranial base, the atlantodental interval (ADI), the clivus length, the clivus-canal angle (CCA)-the angle formed by the clivus and the upper cervical spine, and the basal angle. RESULTS: The mean age of the 100 patients was 50.6 years, and the group included 52 men (52%) and 48 women (48%). In 5 patients (5%), the tip of the odontoid process was more than 2 mm above the Chamberlain line, and in one of these 5 patients (1% of the study group). it was more than 5 mm above it. One patient had a Grabb-Oakes measurement above 9 mm (suggesting ventral cervicomedullary encroachment). The mean ADI value was 1.1 mm. The thickness of the external occipital protuberance ranged from 7.42 to 22.36 mm. The mean clivus length was 44.74 mm, the mean CCA was 153.68° (range 132.32°-173.95°), and the mean basal angle was 113.73° (ranging from 97.06°-133.26°). CONCLUSIONS: The data obtained in this study can be useful for evaluating anomalies of the CCJ in comparison with normal parameters, potentially improving the diagnostic criteria of these anomalies. When evaluating CCJ malformations, one should take into account the normal ranges based on CT scans, with more precise bone landmarks, instead of those obtained from simple plain radiographs.

Intramedullary hemangioblastomas: surgical results in 16 patients.

OBJECT Hemangioblastomas are rare, benign, highly vascularized tumors that can be found throughout the neuraxis but are mainly located in the cerebellum and in the spinal cord. Spinal hemangioblastomas can present with motor and sensory deficits, whose severity varies according to the size and location of the tumor. Resection is the best treatment option to avoid neurological deterioration. The authors report surgical results in the treatment of intramedullary hemangioblastomas and discuss the technical nuances important to achieving total resection without adding new deficits. METHODS A consecutive series of patients with intramedullary hemangioblastomas operated on between 2000 and 2014 by the senior author (H.T.) is presented. The functional scale proposed by McCormick was used to evaluate the patients' neurological status before and after surgery. RESULTS Sixteen patients were included in the study and underwent 17 surgeries. Follow-up was at least 6 months. Age at presentation varied from 13 to 58 years (mean 33.8 years). Ten patients (62.5%) were males and 6 patients (37.5%) were females. Seven (43.75%) of the 16 patients had associated von Hippel-Lindau syndrome, with hemangioblastomas also presenting in other locations. Three patients had multiple tumors in the same segment in the spinal cord, and 10 patients (62.5%) presented with cysts. According to the site of presentation, 11 tumors (68.75%) were localized at the cervical region (including the cervicomedullary junction) and 5 tumors (31.25%) at the thoracic level. Total resection was achieved in all cases, evidenced by postoperative MRI. Four patients had some functional worsening immediately after surgery. After 6 months, 1 patient had functional worsening compared with preoperative status, and 2 patients had clinical improvement. The majority of the patients remained clinically stable postoperatively. CONCLUSIONS Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors, with minimal morbidity and maximum functional recovery. Outcome seems to be directly related to the neurological status before surgery.

Long-term postoperative atrophy of contralateral hippocampus and cognitive function in unilateral refractory MTLE with unilateral hippocampal sclerosis.

OBJECTIVE: This study aimed to evaluate long-term atrophy in contralateral hippocampal volume after surgery for unilateral MTLE, as well as the cognitive outcome for patients submitted to either selective transsylvian amygdalohippocampectomy (SelAH) or anterior temporal lobe resection (ATL). METHODS: We performed a longitudinal study of 47 patients with MRI signs of unilateral hippocampal sclerosis (23 patients with right-sided hippocampal sclerosis) who underwent surgical treatment for MTLE. They underwent preoperative/postoperative high-resolution MRI as well as neuropsychological assessment for memory and estimated IQ. To investigate possible changes in the contralateral hippocampus of patients, we included 28 controls who underwent two MRIs at long-term intervals. RESULTS: The volumetry using preoperative MRI showed significant hippocampal atrophy ipsilateral to the side of surgery when compared with controls (p<0.0001) but no differences in contralateral hippocampal volumes. The mean postoperative follow-up was 8.7 years (± 2.5 SD; median=8.0). Our patients were classified as Engel I (80%), Engel II (18.2%), and Engel III (1.8%). We observed a small but significant reduction in the contralateral hippocampus of patients but no volume changes in controls. Most of the patients presented small declines in both estimated IQ and memory, which were more pronounced in patients with left TLE and in those with persistent seizures. Different surgical approaches did not impose differences in seizure control or in cognitive outcome. CONCLUSIONS: We observed small declines in cognitive scores with most of these patients, which were worse in patients with left-sided resection and in those who continued to suffer from postoperative seizures. We also demonstrated that manual volumetry can reveal a reduction in volume in the contralateral hippocampus, although this change was mild and could not be detected by visual analysis. These new findings suggest that dynamic processes continue to act after the removal of the hippocampus, and further studies with larger groups may help in understanding the underlying mechanisms.

Measuring the impact of the Thoracolumbar Injury Classification and Severity Score among 458 consecutively treated patients.

CONTEXT: The Thoracolumbar Injury Classification and Severity Score (TLICS) was proposed to improve injury classification and guide surgical decision-making of thoracolumbar spinal trauma (TLST), but its impact on the care of patients has not been quantified. STUDY DESIGN: Retrospective study. PATIENT SAMPLE: Analysis of 458 patients treated for TLST trauma from 2000 through 2010 at a single center. Outcome measures Neurological status - ASIA Impairment Scale (AIS), failure of conservative treatment, and surgical complications. METHODS: Clinical and radiological data were evaluated. Patients were grouped according to the period before (2000-2006) and after (2007-2010) utilization of the TLICS. RESULTS: From 2000 to 2006, 148 patients were initially treated conservatively (C) and 66 were surgically (S) treated. In the C group, the TLICS ranged from 1 to 7 (median 1; mean 1.57). In the S group, the TLICS ranged from 2 to 10 (median 2; mean 4.14). The TLICS matched treatment in 97.9% of conservatively treated patients. From 2007 to 2010, 162 patients were initially treated C and 82 were treated S. In the C group, the TLICS ranged from 1 to 4 (median 1; mean 1.48). In the S group, the TLICS ranged from 2-10 (median 4; mean 4.4). The TLICS matched treatment in 98.8% of C-treated patients. Overall, failure of C treatment occurred in nine patients; most failures (7/9) and all three missed distractive injuries occurred prior to use of the TLICS. CONCLUSIONS: After introduction of the TLICS, there was a trend towards more successful conservative treatment with fewer conversions to surgical treatment.

Clinical results of patients with subaxial cervical spine trauma treated according to the SLIC score.

OBJECTIVE: The Subaxial Injury Classification (SLIC) system has been developed to improve injury classification and guide surgical decision making yet clinical validation remains necessary. METHODS: We evaluated the validity and safety of the SLIC system prospectively in patients treated for subaxial cervical spine trauma (SCST) between 2009 and 2012. Patients with four or more points were surgically treated, whereas patients with less than 4 points were conservatively managed. OUTCOME MEASURES: Neurological status was assessed as the primary outcome of successful treatment. RESULTS: Non-surgical group - Twenty-three patients were treated non-surgically, 14 (61%) of them with some follow-up at our institution. Follow-up ranged from 3 to 5 months (mean of 4.42; median 4). The SLIC score ranged from 0 to 6 points (mean and median of 1). One patient with a SLIC of 6 points refused surgery. Surgical group: Twenty-five patients were operated, but follow-up after hospital discharge was obtained in 23 (92%) patients (range from 1 to 24 months, mean of 5.82 months). The SLIC score in this group ranged from 4 to 9 points (mean and median of 7). No patients had neurological worsening. Eight of 13 patients with incomplete deficits had some improvement in American Spinal Injury Association score. CONCLUSIONS: This is the first prospective application of the SLIC system. With regard to our primary outcome, neurological status, the SLIC system was found to be a safe and effective guide in the surgical treatment of SCST.

Upper cervical injuries - a rational approach to guide surgical management.

CONTEXT: The complex anatomy and the importance of ligaments in providing stability at the upper cervical spine region (O-C1-C2) require the use of many imaging modalities to evaluate upper cervical injuries (UCI). While separate classifications have been developed for distinct injuries, a more practical treatment algorithm can be derived from the injury pattern in UCI. OBJECTIVE: To propose a practical treatment algorithm to guide treatment based on injuries characteristic of UCI. METHODS: A literature review was performed on the Pubmed database using the following keywords: (1) "occipital condyle injury"; (2) "craniocervical dislocation or atlanto-occipital dislocation or craniocervical dislocation"; (3) "atlas fractures"; and (4) "axis fractures". Just articles containing the diagnosis, classification, and treatment of specific UCI were included. The data obtained were analyzed by the authors, dividing the UCI into two groups: Group 1 - patients with clear ligamentous injury and Group 2 - patients with fractures without ligament disruption. RESULTS: Injuries with ligamentous disruption, suggesting surgical treatment, include: atlanto-occipital dislocation, mid-substance transverse ligament injury, and C1-2 and C2-3 ligamentous injuries. In contrast, condyle, atlas, and axis fractures without significant displacement/misalignment can be initially treated using external orthoses. Odontoid fractures with risk factors for non-union are an exception in Group 2 once they are better treated surgically. Patients with neurological deficits may have more unstable injuries. CONCLUSIONS: Ascertaining the status of relevant ligamentous structures, fracture patterns and alignment are important in determining surgical compared with non-surgical treatment for patients with UCI.