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Abdominal pseudocysts are common complications of ventriculoperitoneal shunt (VPS). We report the case of a 37-year-old woman treated by VPS for congenital obstructive hydrocephalus, who presented shunt dysfunction related to a voluminous abdominal cyst initially diagnosed as cerebrospinal fluid pseudocyst. The cyst was drained and the VPS was removed after endoscopic third ventriculocisternostomy (ETV). A few months later, a large new abdominal cyst appeared and was operated on. Diagnosis was rectified as massive ovarian mucinous cystadenoma. In any intra-abdominal cyst, differential diagnoses need considering to avoid mis-diagnosis in shunted patients, especially if the cyst is very large. The etiology of the hydrocephalus should also be investigated in case of shunt dysfunction. Even in case of longstanding shunt, ETV can be an alternative to shunt revision surgery in obstructive hydrocephalus, enabling VPS withdrawal and treatment of the hydrocephalus.

Background Idiopathic normal-pressure hydrocephalus (iNPH) is a treatable cause of gait and cognitive impairment. iNPH should be differentiated from ventriculomegaly secondary to brain atrophy to choose the best therapeutic option (ventriculoperitoneal shunt vs medical management). Objective To determine the diagnostic accuracy of automated sulcal morphometry to differentiate patients with iNPH from patients with ventriculomegaly of neurodegenerative origin. Methods Thirty-eight consecutive patients with iNPH (shunt responsive n = 31, nonresponsive n = 7), 35 with vascular cognitive disorder, and 25 age- and sex-matched healthy controls were prospectively included and underwent cognitive evaluation and 3T brain magnetic resonance imaging. Sulcal opening of 10 sulci of interest was retrospectively measured using an automated surface-based approach from the 3-dimensional T1-weighted images. Receiver-operating characteristic curve analyses determined the best parameter to identify iNPH patients. Results The best parameter to discriminate shunt-responsive iNPH from patients with vascular cognitive disorder and healthy controls was the ratio between calcarine sulcus and cingulate sulcus opening with an area under the curve of 0.94 (95% CI: 0.89, 0.99). A cut-off value of 0.95 provided the highest sensitivity (96.8%) and specificity (83.3%). Conclusion This preliminary study showed that automated sulcal morphometry may help the neurosurgeon to identify iNPH patients and to exclude other causes of ventriculomegaly.

OBJECTIVE To determine whether cervical vestibular evoked myogenic potentials (cVEMPs) are predictive of hearing preservation in patients undergoing vestibular schwannoma removal through middle fossa craniotomy approach. STUDY DESIGN Retrospective case study. SETTING Tertiary referral center. PATIENTS Eighteen patients who underwent a middle fossa craniotomy for vestibular schwannoma (stage I or II of Koos classification) with attempted hearing preservation from January 2008 to February 2016 were retrospectively reviewed. INTERVENTION Pre-surgical cVEMPs test, videonystagmography (caloric test), and magnetic resonance imaging (MRI) as well as a pre- and post-surgical audiometry test. MAIN OUTCOME MEASURES cVEMPs parameters including amplitude asymmetry ratio (AR), P13, and N23 latencies and peak-to-peak amplitude between P13 and N23 waves were calculated. Hearing data were classified according to the AAO-HNS hearing classes. The nerve of origin of the tumor was specified during surgery and the largest tumor diameter was measure on MRI axial plane on T2-CISS weighed images. RESULTS Preoperative amplitude asymmetry ratio was lower (n = 15, Mann-Whitney U test, p

Introduction Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center. Materials and methods All recent articles concerning tumors other than vestibular schwannoma in NF2 disease were reviewed, using PubMed databases. Results Intracranial meningiomas occur in 50% of NF2 patients, and are often multiple. Surgery remains the main treatment and should be performed in cases of growing tumors. The role of antiangiogenic therapy is currently under evaluation and the role of radiosurgery still remains to be defined in NF2 disease. Spinal tumors occur in about half of NF2 patients. Surgery should be discussed when radiological tumor progression is demonstrated, even if spinal tumors are asymptomatic, in order to preserve neurological function and good quality of life. As regards lower cranial nerve schwannomas, radiosurgery appears to be a more appropriate treatment for growing tumor with a small volume in order to avoid post-operative complications, especially swallowing disorders. Facial nerve schwannomas may appear, on MRI, like vestibular schwannomas. The diagnosis should be suspected when the facial palsy is an early symptom during cerebello-pontine tumor progression. Trigeminal schwannomas are frequent in NF2 disease and fortunately they are often asymptomatic. Among major neurofibromatosis types, peripheral nerve sheath schwannomas are only present in patients with NF2 disease and schwannomatosis. Surgical resection is required when the cutaneous schwannomas is painful or when tumor progression is observed and causes symptoms. Conclusion Tumors other than vestibular schwannoma are also associated with a poor prognosis in NF2 patients. Surgery remains the main treatment in most cases. Each treatment decision in NF2 disease requires a complete evaluation of all cranial and spinal locations of the disease in order to establish surgical priorities and strategies.

The challenge of the neurosurgical management of gliomas lies in achieving a maximal resection without persistent functional deficit. Diffusion tensor imaging (DTI) allows non-invasive identification of white matter tracts and their interactions with the tumor. Previous DTI validation studies were compared with intraoperative cortical stimulation, but none was performed based on the tumor anatomopathological analysis. This preliminary study evaluates the correlation between the preoperative subcortical DTI tractography and histology in terms of fiber direction as well as potential tumor-related fiber disruption. Eleven patients harboring glial tumors underwent preoperative DTI images. Correlations were performed between the visual color-coded anisotropy (FA) map analysis and the tumor histology after “en bloc” resection. Thirty-one tumor areas were classified according to the degree of tumor infiltration, the destruction of myelin fibers and neurofilaments, the presence of organized white matter fibers, and their orientation in space. After histologic comparison, the DTI sensitivity and specificity to predict disrupted fiber tracts were respectively of 89% and 90%. The positive and negative predicted values of DTI were 80% and 95%. The DTI data were in line with the histologic myelin fiber orientation in 90% of patients. In our series, the prevalence of destructed fiber was 31%. Glioblastoma WHO grade IV harbored a higher proportion of destructed white matter tracts. Lower WHO grades were associated with higher preservation of subcortical fiber tracts. This DTI/histology study of “en bloc”–resected gliomas reported a high and reproducible concordance of the visual color-coded FA map with the histologic examination to predict subcortical fiber tract disruption. Our series brought consistency to the DTI data that could be performed routinely for glioma surgery to predict the tumor grade and the postoperative clinical outcomes.

International audience; BACKGROUNDHearing response following an osteodural decompression of the internal auditory canal (IAC) is controversial.OBJECTIVETo evaluate the course of auditory brainstem responses (ABRs) and the early hearing response during the first year following IAC decompression for small to medium-sized vestibular schwannomas occurring in neurofibromatosis type 2 (NF2).METHODSRetrospective chart review of middle fossa craniotomy for IAC osteodural decompression in NF2-related vestibular schwannomas.RESULTSTwelve NF2 patients were operated on from 2011 to 2016 for IAC decompression. All had NF2 according to the Manchester criteria. All had a progressive change of their ABRs documented from the diagnosis of NF2 over a mean period of 6.25 [0.36;10.9] yr. Treatment was proposed to stop hearing progression based on the speech discrimination scores (SDSs; n = 4) or for hearing maintenance (n = 8). In patients with prior hearing progression, hearing responses were observed in 3 of the 4 patients during the first year. One patient kept on progressing. In the hearing maintenance group, the SDSs remained stable. SDSs improved from 85% [20-100] to 92.5% [60-100] on average (n = 12) and from 55% [20-80] to 77.5% [50-100] in the hearing progression group (n = 4). ABRs improved in 4 patients following decompression.CONCLUSIONIAC decompression allows early objective hearing responses in select patients. We suggest that the procedure should be offered to patients with hearing progression based on their SDSs and/or associated progressive increases in their wave III and V latencies on ABRs.

Resume L’hydrocephalie chronique de l’adulte se caracterise par une triade clinique associant des troubles de la marche, une deterioration neuro-cognitive et une incontinence urinaire. Les signes apparaissent de facon insidieuse chez un patient de plus de 40 ans. Le diagnostic radiologique est fait sur une IRM cerebrale qui retrouve un elargissement ventriculaire, des anomalies de la repartition du liquide cerebro-spinal (LCS) des espaces sous-arachnoidiens (elargissement des scissures laterales mais rarefaction du LCS au vertex) et un flux du LCS aqueducal augmente ; en cas d’hydrocephalie obstructive sur une stenose decompensee de l’aqueduc, ce flux est nul et le plancher du troisieme ventricule est abaisse. Des tests complementaires (PL depletive d’au moins 40 mL de LCS ou un test de perfusion) permettent de confirmer l’indication chirurgicale a une derivation ventriculo-peritoneale (en cas d’hydrocephalie communicante) ou a une ventriculo-cisternostomie (en cas d’hydrocephalie obstructive). Une amelioration clinique est retrouvee chez 80 % des patients si l’indication chirurgicale est bien posee, faisant de cette pathologie une des seules causes de demence curable.

Internal variation among spheno-orbital meningiomas (SOM) is surgically challenging. Optic canal invasion management is discussed. This retrospective study includes 70 patients with SOM who underwent surgery between 1995 and 2012. Preoperative ophthalmological, neurological and aesthetic clinical signs were collected. All patients benefitted from repeated tomography and magnetic resonance imaging (MRI). The surgical team consisted of a neurosurgeon and a plastic surgeon. In the majority of cases, resection was followed by bone reconstruction using an autologous iliac crest graft. The extent of resection was evaluated on the dural and osseous sides. Early clinical outcomes, long-term follow-up, recurrence and adjuvant therapies were reported. The mean age was 52 years old, and 91 % of the patients were women. Initial symptoms primarily included proptosis (65 %), decreased visual acuity (39 %) and soft tissue tumefaction (16 %). We classified 40 cases as the internal variety when considering the inner third of the greater wing of the sphenoid, optic canal, anterior clinoid process or cavernous sinus. The remaining cases were described as the external variety. The complete resection rates for the internal and external varieties were 12 % and 61 %, respectively (P

Introduction Le defi de la prise en charge neurochirurgicale des tumeurs gliales consiste a realiser une ablation tumorale aussi complete que possible sans deficit fonctionnel postoperatoire. La technique de tenseur de diffusion (DTI) permet l’identification non-invasive des fibres de substance blanche et leurs rapports avec la tumeur. Cependant, il reste soumis a de nombreuses limitations techniques et sa fiabilite en tant qu’outil de planification pre- et peroperatoire est remise en question. La plupart des etudes de validation ont ete realisees en comparaison avec la stimulation corticale peroperatoire, mais aucune n’a ete realisee sur l’analyse anatomopathologique de la tumeur pour evaluer l’integrite des fibres de la substance blanche dans l’environnement tumoral. L’objectif de l’etude est donc d’evaluer la faisabilite d’une correlation anatomo-radiologique entre le DTI et l’analyse histologique des tumeurs gliales. Materiel et methodes 12 patients atteints de tumeurs gliales de grade II a IV ont beneficie d’une IRM avec sequence de DTI preoperatoire. Les fibres de substance blanche detruites et infiltrees ont ete identifiees a l’aide de la carte d’anisotropie fractionnelle a codage colorimetrique directionnel (carte RVB) par evaluation visuelle. Les correlations DTI et histologiques ont ete realisees sur 31 regions, classees selon le degre d’infiltration tumorale, la destruction des fibres de myeline et des neurofilaments, la presence de fibres de substance blanche organisee et leur orientation dans l’espace. Resultats Les correlations observees entre le DTI et les analyses histologiques concernant l’orientation et la destruction des fibres sont elevees. Selon le niveau de myeline intacte evalue a l’aide de l’histologie et des fibres detectees sur les images DTI, une concordance est observee pour 23 a 26 des 31 zones etudiees. La concordance directionnelle des faisceaux est egalement observee chez 9 patients sur les 10 presentant des fibres organisees en DTI. Conclusion Cette etude a demontre qu’il est possible d’obtenir une correlation entre les images DTI et l’etude histologique des fibres de myeline dans les tumeurs gliales et a confirme qu’une analyse visuelle de la carte RVB peut etre une methode utile pour evaluer l’infiltration de substance blanche dans les tumeurs gliales. Cependant, d’autres etudes sont necessaires pour evaluer la correlation avec les donnees cliniques afin de determiner le seuil de destruction auquel le faisceau perd sa fonction ( Fig. 1 ).

International audience; BACKGROUND: Ruptured arteriovenous malformations (rAVM) are life-threatening diseases.OBJECTIVE: To evaluate the outcome of patients with grade 1 SPM rAVM after microsurgical treatment.METHODS: We retrospectively included 64 consecutive operated patients with a grade 1 SPM rAVM in our institution between 2002 and 2012. Complications related to the surgical procedure were recorded. All patients were re-evaluated 3months after treatment using the modified Rankin Scale score (mRS). Persistent neurological disorders were evaluated 1year after bleeding. Conventional cerebral angiography was performed for each patient immediately after surgical treatment and 1year later.RESULTS: The mean age at diagnosis was 30.8 years. Initial WFNS score was grade 1 in 25 patients, grade 2 in 11 patients, grade 3 in 10 patients, grade 4 in 9 patients and grade 5 in 9 patients. No remnant was left and a new surgery was performed only in a single patient who was initially operated-on under emergency conditions with limited preoperative investigations due to a poor clinical grade. Early postoperative complications related to the surgical procedure were recorded in 7 patients. The mRS score 3months after treatment was ≤2 in 53 patients (83%). Persistent neurological disorders were recorded in 40 patients (62.5%). High initial WFNS score (>2) and the hydrocephalus were significantly associated (P2).CONCLUSIONS: Grade 1 rAVM is a life-threatening disease concerning in most cases young patients. Long-term morbidity is often related to the hemorrhagic brain damage and rarely to the AVM resection.

International audience; Purpose. - Medial lobe temporal structures and more specifically the hippocampus play a decisive role in episodic memory. Most of the memory functional magnetic resonance imaging (fMRI) studies evaluate the encoding phase; the retrieval phase being performed outside the MRI. We aimed to determine the ability to reveal greater hippocampal fMRI activations during retrieval phase. Materials and methods. - Thirty-five epileptic patients underwent a two-step memory fMRI. During encoding phase, subjects were requested to identify the feminine or masculine gender of faces and words presented, in order to encourage stimulus encoding. One hour after, during retrieval phase, subjects had to recognize the word and face. We used an event-related design to identify hippocampal activations. Results. - There was no significant difference between patients with left temporal lobe epilepsy, patients with right temporal lobe epilepsy and patients with extratemporal lobe epilepsy on verbal and visual learning task. For words, patients demonstrated significantly more bilateral hippocampal activation for retrieval task than encoding task and when the tasks were associated than during encoding alone. Significant difference was seen between face-encoding alone and face retrieval alone. Conclusions. - This study demonstrates the essential contribution of the retrieval task during a fMRI memory task but the number of patients with hippocampal activations was greater when the two tasks were taken into account. (C) 2018 Published by Elsevier Masson SAS.

The causes of mortality in neurofibromatosis type 2 (NF2) patients are poorly studied in the literature. Our study aimed to fit this gap by analyzing the main causes of death in this population.This study is the retrospective review of prospectively collected data of 80 patients with NF2 disease followed in Lille University Hospital between 1987 and 2011. Demographical data, diagnosis criteria, and cause of death were recorded.There were 45 men and 35 women, with a mean age at diagnosis of 27.2 years (range: 6-73 years; SD: ± 15.4). Sixty-eight patients met Manchester criteria and the others had an identified mutation in the NF2 gene which confirmed the diagnosis. Of all patients, we noted 7 deaths. The mean age at diagnosis of dead patients was 26 years. The mean age of death was 38.9 years. The causes of death were suicide in 1 patient, hematoma after surgical removal of grade IV vestibular schwannoma in 1 patient, aspiration pneumonia after swallowing disturbances in 3 patients, intracranial hypertension related to growth of multiple meningiomas in 1 patient, and brachial plexus sarcoma grade 3 in the last patient.NF2 is a serious disease that can quickly be life-threatening. The presence of lower cranial nerves schwannomas is a poor prognostic factor, and radiosurgery should be considered for their treatment, as surgical removal often worsens the swallowing disturbances. A psychological support should also be provided.

Paediatric low-grade astrocytomas of the fourth ventricle are rare tumours, generally revealed by hydrocephalus. However, some patients present with a history of severe anorexia. It might be a harbinger, which if recognized, could lead to earlier diagnosis. We decided to examine our database in order to evaluate the incidence and signification of anorexia in this context. Retrospective monocentric study of cases of low-grade astrocytomas of the fourth ventricle operated between 1991 and 2012 in our paediatric neurosurgery department. We particularly observed the clinical presentation and long-term clinical, oncological and radiological evolution. Non-parametrical tests were used (Mann–Whitney, Fisher). We reviewed 34 cases, 31 pilocytic astrocytomas and 3 diffuse astrocytomas, 16 boys and 18 girls, (M/F ratio 0.89). Mean age at diagnosis was 8 years old. Seven presented with notable anorexia, the average BMI in this group was ≤2 standard deviation (SD); with clinical signs evolving for 11.5 months. Twenty-seven children had no anorexia; average BMI in this group was +1 SD, with clinical evolution for 6 months on an average of p

Resume Introduction Le but de cette etude etait d’analyser l’epidemiologie, le traitement et le pronostic des hemangioblastomes (HGB) du systeme nerveux central (SNC) diagnostiques dans le cadre de la maladie de von Hippel-Lindau (VHL). Methode Nous avons revu une serie de 19 patients traites dans notre service (1996–2008). L’analyse a porte sur les caracteristiques cliniques et radiologiques pre- et postoperatoires, la recherche de lesions peripheriques, le pronostic clinique (modified Rankin scale [mRS], score de McCormick) et l’evolution a long terme. Resultats La symptomatologie revelatrice etait frequemment l’hypertension intracrânienne pour les lesions cerebelleuses (7/15) et un deficit sensitivo-moteur pour les lesions de la moelle allongee (2/5) ou de la moelle spinale (5/11). Les sites preferentiels etaient cerebelleux (15/31) ou spinaux (11/31). Des localisations multiples et des lesions viscerales etaient retrouvees chez deux tiers des patients. L’exerese etait complete dans plus de trois quarts des cas. La mortalite postoperatoire etait de 16 %. Le mRS final etait ≤ 1 dans 17 des 20 localisations infratentorielles et le score de McCormick final ≤ 2 dans toutes les localisations spinales. A distance, deux tiers des patients presentaient une recidive clinique ou une nouvelle localisation evolutive. Conclusion La maladie de VHL doit etre evoquee lorsqu’un HGB survient avant 30 ans, qu’il est de localisation spinale, qu’il est associe a d’autres localisations du SNC ou a des lesions peripheriques typiques.

L’hydrocephalie chronique de l’adulte se caracterise par une triade clinique associant des troubles de la marche, une deterioration neurocognitive et une incontinence urinaire. Le diagnostic radiologique est fait sur une IRM cerebrale qui retrouve un elargissement ventriculaire, des anomalies de la repartition du liquide cerebro-spinal (LCS) des espaces sous-arachnoidiens (elargissement des scissures laterales mais rarefaction du LCS au vertex), un angle calleux diminue et un flux du LCS aqueducal augmente dans les hydrocephalies communicantes ; en cas d’hydrocephalie obstructive sur une stenose decompensee de l’aqueduc, ce flux est nul et le plancher du troisieme ventricule est abaisse. Des tests complementaires (ponction lombaire depletive d’au moins 40 mL de LCS, drainage lombaire externe ou test de perfusion mesurant un index de resistance a l’ecoulement du LCS) permettent de confirmer au cas par cas, lors d’une discussion pluridisciplinaire associant un neurologue, un neuroradiologue et un neurochirurgien, une indication a un traitement chirurgical ; le poids des eventuelles co-morbidites sera discute collegialement. En cas d’hydrocephalie communicante, c’est une derivation ventriculoperitoneale (DVP) ou ventriculo-atriale (DVA) qui sera indiquee. L’intervention dure une heure environ, sous anesthesie generale ; un catheter ventriculaire est insere dans l’atrium ventriculaire ou la corne frontale puis est connecte a une valve qui regulera le debit ou la pression d’ouverture du systeme. Le catheter distal se termine dans la cavite peritoneale (DVP) ou dans l’oreillette droite (DVA). Les risques sont moderes (infection, hematome intracrânien ou malposition du materiel). En cas d’hydrocephalie obstructive, c’est une ventriculocisternostomie endoscopique qui sera proposee. L’amelioration clinique survient dans les semaines qui suivent l’intervention chez plus de 80 % des patients. Le suivi est clinique et radiologique, avec des radiographies de valve verifiant le bon positionnement du materiel et une IRM qui montre habituellement une legere diminution de la taille des ventricules.

Grade 2 meningiomas are a real problem in therapeutic management because of their tendency to reoccur. The most effective treatment is surgery. The role of adjuvant radiotherapy in this disease is still disputed due to its uncertain effect on progression-free survival. To show that early adjuvant radiotherapy is an effective treatment in patients with grade 2 meningiomas. A retrospective study was performed on all patients operated on for grade 2 meningioma in our center between 1994 and 2011. For every patient, we recorded the age at diagnosis, sex, background of neurofibromatosis type 2 (NF2) or meningiomatosis, location of meningioma, quality of tumor resection and whether the patient received early postoperative radiotherapy. These prognosis factors were studied using statistical tests. We included 167 patients (94 women, 73 men, sex ratio = 1.28); the mean age at diagnosis was 53.8 years. Twenty-seven patients received early adjuvant radiotherapy after surgery. Patients who received early postoperative radiotherapy had a significantly longer progression-free survival (8.2 years) than patients without radiotherapy (5.7 years, p = 0.04). In multivariate analysis, quality of tumor resection and early postoperative radiotherapy decrease the risk of recurrence of meningioma (p

In spite of the few clinical studies regarding the occurrence of intracranial meningiomas, their prognosis in neurofibromatosis type 2 (NF2) has not been accurately assessed and their management remains controversial. This study aims to compare NF2 patients with intracranial meningiomas to those without, and consequently to identify prognostic factors in attempt to improve the management of these tumors.This retrospective study includes a total of 80 NF2 patients followed at Lille Hospital Center between 1987 and 2011. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both location and size of each tumor were reported.The mean follow-up period was 8.8 years (range 1-24 years; SD: ±0.8) and the mean age at diagnosis was 27.2 years (range 6-73 years; SD: ±1.7). Among all patients, 34 harbored intracranial meningiomas. Patients with intracranial meningiomas had a higher number of intracranial schwannomas, spinal tumors and cutaneous tumors (p0.05). They underwent more surgical procedures (p0.012). Twenty five intracranial meningiomas were surgically removed in 17 patients. The decision to perform surgery was taken in 10 cases for symptomatic tumors and in 15 cases for growing asymptomatic tumors determined by radiology. The histological analysis found a high rate of fibroblastic, transitional or grade 2 meningiomas preferentially located at the cerebri falx.Intracranial meningiomas are common in NF2. They are associated with poor prognosis factors. Clinical and radiological monitoring could lead to early treatment of these tumors both when clinical symptoms are present and in case of proven radiological evolution, and thus trying to maintain a favorable functional prognosis for as long as possible.

Non-syndromic coronal synostoses oxycephaly and brachycephaly (NSCSOB) are rare. Their natural history, surgical management, and outcome are debated. We reviewed the available literature on NSCSOB. In addition, we reviewed retrospectively our experience with cases of NSCSOB, managed and operated by our joint craniofacial team since 1984. Newborns underwent perifrontal craniectomy, while infants and older children underwent fronto-orbital advancement with frontoparietal remodeling. Cases with delayed onset of faciosynostosis were excluded. Some cases of NSCSOB present at birth or even prenatally with gross dysmorphism and severe brachycephaly; others present later in life with harmonious restriction of intracranial volume and are at risk for development and vision. Some NSCSOB evolve from initially unisutural synostoses. We treated 61 cases, operated aged 1.4 to 106.7 months, 30 with brachycephaly and 31 with oxycephaly. Twelve of these (19.7 %), had been treated initially for sagittal or unicoronal synostosis, and evolved into NSCSOB. Intracranial hypertension was present initially in 28 (45.9 %), with ophthalmological consequences in 13 (21.3 %) and mental retardation in 6 (9.6 %). No patient had hydrocephalus; only two had asymptomatic Chiari malformation. The mean postoperative follow-up was 113.6 months. At last control, 21 patients had developmental delay and 5 had visual impairment. NSCSOB are a complex entity. They have in common a high risk of severe intracranial hypertension and rare hydrodynamic complications. Genetic screening is necessary to assert their non-syndromic nature. Prolonged follow-up of all craniosynostoses is necessary because unisutural synostoses can evolve into NSCSOB, and NSCSOB can evolve into craniofaciosynostosis.

Introduction The outlook of pediatric hydrocephalus has spectacularly improved over the past decades; however, the adult outcome is still poorly documented. Determining the healthcare profile of these patients is important in order to organize the management of this growing population. We decided to review our pediatric hydrocephalus database for pediatric patients treated for hydrocephalus and followed up into adulthood. Methods Our institution has a virtual monopoly for pediatric hydrocephalus, serving a four-million-plus population; the transition to adult care is also managed in the same institution. We retrospectively reviewed patients younger than 18 treated for hydrocephalus since 1980 and followed up beyond the age of 20. Results We reviewed 456 patients, with a mean initial age of 55.6 months, and followed up for a mean of 24.2 years. In 81 patients (17.8%), the last shunt operation occurred after 20 years; 22 of these (4.9% of the total) having never been revised earlier. Sixteen patients (3.5%) could be weaned of their shunt. Thirteen patients died in adult age, 5 of these dying of shunt-related causes. The most prominent sequels were motor (46.5%) and cognitive (47.6%); only 82 patients (18.0%) had no sequel at all. Intelligence quotient values were ≥80 in 54.5% of tested patients; however, schooling was normal in only 41.4%, and only 33.7% was employed in the competitive labor market. Conclusion Adults treated for hydrocephalus in childhood require a life-long follow-up. Late mortality is low but not null, morbidity is high, and many patients require shunt surgery during adulthood. The transition from child to adult neurosurgery needs to be organized for these vulnerable patients.

Background and purpose Haemophilia is a well-known X-linked recessive bleeding disorder related to a deficiency of factor VIII. The aim of the paper is to point out the role of an early diagnosis in cases of intracranial bleeding, especially in neonates and to highlight the bad prognosis. Methods Three patients were included in the study from our prospective data. All had severe factor VIII deficiency (less than 1%). Results The first of them was a newborn without familial history of haemophilia which presented with hypotonia and seizures after a delivery with vacuum. CT showed a subdural haematoma and MRI multiple ischemic lesions. Two months later, he died from a new intracranial haemorrhagic episode resistent to infusion of factor VIII related to development of an anti-factor VIII antiboby. The second neonate had no familial history more and experienced hypothermia and areactive mydriasis because of an acute subdural haematoma. He died eight days later of ischemic lesions in spite of a craniotomy. The third was a 5-month-old boy with a familial history. Physical examination revealed lethargy, seizure and a recent cranial perimeter increase. He underwent a craniotomy with a good result. Conclusion The authors stress the need for immediate factor VIII infusion and appropriate neurosurgical work-up. The rapid medical management is mandatory if morbidity and mortality are to be minimized.

The lateral ventricles are the C-shaped cavities of the telencephalon. Embryology of theses cavities is recalled as well as the immediate relationship of the frontal horn, the body, the atrium and the temporal and occipital horns.

Central neurocytoma is the second most frequent tumor in our series of 284 lateral ventricle tumors (12%). Mean age was 32.6 years. There was a male predominance (20/35). Most frequent clinical signs were neuropsychological disturbances and intracranial hypertension. On imaging, these tumors concentrate central calcifications in more than half of cases. Main locations were frontal horn and corpus of lateral ventricle (82%). Uneventful postoperative course was recorded in 52% of cases. Immunoreactivity against neuron-specific enolase (NSE), synaptophysin, S 100 protein, Microtubul Associated Protein de type 2 (MAP 2) and calcineurin confirms the neuronal nature of the neoplasm. This tumor has a good prognosis. Local control after surgery is obtained in 68% of cases. Gamma knife surgery seems to be useful in cases of little volume with tumoral remnants or recurrence, when radiological appearance is well circumscribed and round-shaped.

Twenty-seven cases of ependymoma were collected in the French multicentric study dedicated to lateral ventricle tumors. The mean age of patients was 31,5 years. All patients underwent surgery, with complete resection of the tumor in 76% of cases. Histologic examination disclosed 23 grade 2 ependymomas and four grade 3 ependymomas. Postoperative mortality was 7%. Four patients underwent radiotherapy, and five chemotherapy. Local recurrence of the tumor occurred in seven cases, with a mean delay of 48 months. Disseminated disease within the central nervous system was found in only one patient. Median survival in all the 27 patients was 181 months, and median progression-free survival was 106 months. The relevant series of the literature are reviewed in order to precise the prognostic factors of ependymomas and therapeutic strategies.

Subependymoma is a benign lesion, slow-growing neoplasm, representing 0.2 to 0.7 % of intracranial tumors. They are often clinically silent, incidentally discovered at autopsy. These symptoms are related to big volume. They are attached to the septum pellucidum, leading to hydrocephalus by Monro foramen obstruction. Overall mean age at diagnosis is 39 years with more males than females. At CT-scan, subependymoma shows a slightly low attenuation compared to gray matter. There is no or mild enhancement following contrast injection. On MR T1-weighted imaging, subependymoma is isointense and hyperintense on MR T2-weighted imaging. Intramural calcifications and cystic components are noted in 20 to 30 % of patients. Peritumoral oedema is absent. Immunohistochemicals studies show intense positivity for S-100 and GFAP. The treatment is surgical with an excellent prognosis.

The lateral ventricle is a deep-seated cavity, overlayed by a cortical mantle which contains eloquent areas, especially on the dominant hemisphere, and surrounded by the optic radiations. The surgical approach requires a thorough preoperative reflexion based on magnetic resonance imaging, in order to understand the site of origin and the vascular pedicles of the tumor. Surgical approaches to the frontal horn, temporal horn and atrium are successively described.

The data from 284 cases of lateral ventricle (VL) tumors treated in 18 departments of Neurosurgery in France between 1995 and 2010 were collected in this series. The mean age was 33 years with a large proportion of children (79/284). Clinical presentation The mean duration of symptoms was short in time (2 months). The revelation was incidental in 10% of the cases ; for symptomatic lesions, raised intracranial pressure (ICP, due to hydrocephalus) and neuropsychological signs were the most frequent. Only one third of the patients had no CSF disorder. Histopathology The two most frequent tumors were meningiomas (40 cases, 15%) and neurocytomas (35 cases, 13%). The sub-ependymal giant cells astrocytomas, the ependymomas and sub-ependymomas were also common (11, 10 and 9% respectively). Therapeutic management A surgical procedure was performed in 258 patients, with the help of neuronavigation in one third of cases. The patients were most frequently operated with a transfrontal approach (40%). The resection was complete in 73%, which was a statistical factor of global survival. The postoperative mortality rate was 4.9%. Outcome Neuropsychological deficits were the most frequent sequelae (22.8%) due to raised ICP. Campimetric deficits (19%), seizures (19%), hemiparesis (15%) and language disorders (12%) were then found. The mean follow-up was 52 months with good evolution for 58% of patients. Fifteen percent of patients had a handicap and, finally, 12.9% will die. At last, two third of living patients had no tumoral residue or re-evolution.

The lateral ventricle (LV) has a deep position within the cerebral hemisphere. The LV is covered by white matter with important functional role in the dominant hemisphere. Lateral wall of the frontal horn is covered by the inferior occipitofrontal fasciculus (IOFF) and its roof by the corpus callosum (CC). The body of the LV has the same cranial relationship and is covered laterally by fibers of internal capsula and arcuate fasciculus; its lower part is in relationship with the body of the fornix. The atrium of the LV is covered by the arcuate fasciculus and its lower part is covered by the IOFF and optic radiations. The inferior horn or temporal horn is covered by optic radiations in depth of middle temporal gyrus (T2). The auditive radiations crossed the optic radiations at the level of the roof of the inferior horn.

Tumors of the lateral ventricle are not as rare in children as in adults and present complex problems which are to be dealt with often in a context of emergency, on account of their large size and the risk of life-threatening bleeding in a small child. Their diagnostic features are different in children, especially in infants and even more so in the fetus. The main surgical problems are the risk of bleeding and of hydraulic complications. From an oncological perspective, the main tumor types are choroid plexus tumors, subependymal giant-cell astrocytoma, and low-grade gliomas. The developmental outcome of these patients is often poor, on account of severe intracranial hypertension and young age at diagnosis.

Metastases of lateral ventricle (LV) are attached to choroidal plexus. Primary source is cancer of kidney. Two thirds of patients are male. Oligodendrogliomas occur in young females. Signs of increased intracranial pressure are a constant feature. These tumors are found in anterior portion of the LV with severe enhancement and clumped calcifications. Treatment is surgical. Cavernomas have a preponderance of rapid growth with a bleeding revelation. Seizures are rare. Rebleeding is frequent and justifies a surgical treatment. Schwannomas are a rare entity in which the majority of patients are very young. MRI shows calcifications, cystic components and a strong enhancement. They are limited to sporadic cases, never associated with neurofibromatosis. Arachnoid cysts are located in the atrium and/or in the occipital horn. Patients are young (mean age < 40 years). MRI demonstrates an intracystic lesion with signal intensity similar to the CSF. Best treatment is an endoscopic fenestration. Epidermoid cysts occur in third decade. These pearly tumors appear isointense or a little hyperintense on T1-weighted imaging, very characteristic. They are enhanced after gadolinium injection and appear strongly hyperintense on T2-weighted imaging. An incomplete removal with a thorough long-term follow-up is necessary. Cavernomas of LV are hyperintense on T1- and T2-weighted imaging. They have a bleeding risk of 25 to 45%. Therefore, they must be operated.

Tumors of the frontal horn of the lateral ventricle (LV) are only supplied by the posteromedial choroidal artery. Tumors of the body of the LV are supplied by the same artery. Tumors of the atrium of the LV with anterior extension are supplied by both posteromedial choroidal and posterolateral arteries. Tumors of the atrium with inferior extension are supplied by both anterior choroidal artery and posterolateral choroidal arteries. Tumors of the inferior horn are only supplied by anterior choroidal artery. The tumoral venous drainage is organized with three main groups of veins: a medial group, a lateral group and a choroidal group.

This series reports 40 cases of meningiomas of the lateral ventricle treated in France between 1995 and 2010. The mean age was 52 years. The revelation was incidental in 23% of cases. 36 patients underwent surgery with uneventful postoperative course in 75% of them. Visual field deficits were the most frequent sequelae. In six cases, recurrences occurred during the follow-up period. The relevant series of the literature were discussed in order to precise the therapeutic strategies and the outcome.

Meningiomas of the cerebellopontine angle (CPA), although homogenous in terms of location, present different surgical challenges depending on their site of dural origin and extension. Complete tumor resection sometimes leads to high morbidity. The objective of this work is to evaluate the results of surgery and the place of additional treatments. In a series of 115 patients with CPA meningiomas, we retrospectively studied 69 patients operated on between 1994 and 2007 using a retrosigmoid approach. Clinical presentation, operative findings and functional outcome were reviewed for a mean follow-up time of 36 months. Usual presentation was hearing loss associated with gait disturbance (50%) and vertigo (35%). Preoperative cochlear evaluation was abnormal in 40% of the cases. Most tumors (90%) originated on the posterior face of the petrous part of the temporal bone. In one-third of the cases, the meningioma had invaded the internal acoustic meatus. Total or subtotal tumor removal was achieved in 91% of patients without perioperative mortality. Fourteen patients received additional treatment with radiotherapy or radiosurgery for a small residual tumor, often deliberately left in place to reduce operative morbidity. At long-term follow-up, facial nerve function was preserved in 91% of the cases. Hearing worsened in 17% of patients. The tumor recurred in only one case. The retrosigmoid approach is a safe surgical procedure. The modern management of CPA meningiomas should achieve as complete a resection as possible within reasonable limits, considering that a small residual tumor can be controlled fairly easily with radiosurgery for a relatively long time.

Glioblastomas (GBMs) are highly malignant tumors characterized by microvascular proliferation and the pseudopalisading pattern of necrosis. Investigations have, therefore, focused on vascular and endothelial cell biology in GBM. Endocan, also called endothelial cell-specific molecule-1, is a proteoglycan that is secreted by endothelial cells and upregulated by proangiogenic factors. We found that endocan is not only expressed in vitro by endothelial cells but also in the T98G and U118MG human GBM cell lines. In U118MG cells, tumor necrosis factor and fibroblast growth factor 2 upregulated endocan production, whereas exposure to hypoxia or cobalt chloride, an inducer of hypoxia inducible factor 1, increased endocan release without affecting cell viability. Endocan expression in 82 brain tumors was studied by immunohistochemistry. Endocan immunoreactivity was detected in hyperplastic endothelial cells in high-grade gliomas, mostly at the tumor margins; endothelial cells were mostly endocan negative in low-grade gliomas, and it was never detected in the cerebral cortex distant from the tumors. Tumor cells in high-grade but not low-grade gliomas also expressed endocan, and it was detected in palisading cells surrounding areas of necrosis in GBM. Endothelial cell endocan immunoreactivity also correlated with shorter survival in glioma patients. Taken together, these results suggest that endocan is associated with abnormal vasculature in high-grade gliomas.

Background Anti-Human Epithelial Receptor Type 2 (HER2) antibodies have the ability to induce in vitro apoptosis of glioblastoma (GBM) cells. This study was designed to evaluate the variability of HER2 expression in GBM and its role as a possible prognosis factor. Methods Data of 57 patients with GBM and 16 patients with grade III gliomas were retrospectively analyzed. The expression of HER2 was determined by immunohistochemistry and intensity was noted from 0+ to 3+. We compared the HER2 expression in de novo GBM and in GBM resulting from anaplastic transformation of lowgrade glioma (‘‘secondary GBM’’). Statistical analysis was performed using univariate analysis and the Kaplan–Meier method. Findings All GBM expressing highly HER2 (2+ and 3+) were de novo GBM. All secondary GBM expressed HER2 with low intensity (0+ and 1+). Survival time was significantly longer when HER2 expression was low (Log Rank test P = 0.04). The patterns of HER2 expression were similar between grade III gliomas and secondary GBM. Conclusions To our best knowledge, our study showed for the first time a significant association between HER2 expression and the type of GBM, with subsequent influence on survival rate. GBM with low-HER2 expression are more likely to be secondary GBM, carrying a better prognosis than de novo GBM.

Vestibular schwannoma (VS) growth in neu- rofibromatosis type 2 (NF2) can be responsible for brain- stem compression and hearing loss. Surgical removal remains the standard therapy despite potential morbidity. Previous studies suggested that the inhibition of the VEGF- pathway with bevacizumab could result in hearing im- provement, reduction of the tumor volume or both in adults. We retrospectively describe the French experience of bevacizumab treatment delivered for progressive VS in pediatric NF2 patients. Patients received Bevacizumab 5 or 10 mg/kg every 2 weeks according to the physician's choice. Follow-up included clinical assessment, au- diometry and volumetric MRI every 3-6 months. Seven patients harboring 11 VS were included. The median age at inclusion was 15 years (11.4-18.8), and the median treat- ment duration was 11.3 months (3.2-55.6). At baseline, the median tumor volume was 1.2 cm 3 (0.52-13.5) and the median word recognition score was 90 % (0-100). We observed one major response, two minor responses and a decrease in the rate of tumor growth for the 4 other pa- tients. The median annual growth rate before treatment was significantly higher than after 1 year of treatment (138 vs. 36 %, n = 5, p = 0.043). We noted one hearing im- provement over the course of 1 year under treatment (hearing response rate was 14 %). Overall, the treatment was well tolerated. Our study supports that bevacizumab is an attractive therapeutic option for pediatric NF2 patients with growing VS. Thorough multidisciplinary evaluation is necessary to identify the best candidates prior to treatment. It is likely that a better functional outcome would be ex- pected if targeted therapies were discussed early in the management of the disease.

International audience; OBJECTIVE: Our study aimed to evaluate the functional outcome and the risk of postoperative remnant in patients with rAVM after microsurgical treatment.METHODS: This is a retrospective of 139 consecutive patients operated for a rAVM between 2002 and 2012 in our institution. The age at diagnosis and the WFNS score were recorded for each patient before treatment. All patients were re-evaluated 3 months after treatment using mRS scale. Conventional angiography was performed in the first 2 postoperative weeks and then a year later to detect any remnant or recurrence.RESULTS: The mean age at diagnosis was 30.8 years (range 4-69 SD: ±5) and 44 patients had an age at diagnosis 2, SPM grade>2 and preoperative evaluation limited only to CT angiography in emergency situation (p

Journal of Neuroradiology - In Press.Proof corrected by the author Available online since jeudi 2 fevrier 2012

The authors report the case of a 38-year-old patient who presented with a ruptured anterior spinal artery aneurysm confirmed by MRI. This patient harbored neurological deterioration related to a rebleeding at Day 1. Control angiography showed spontaneous occlusion of both aneurysm and distal anterior spinal axis. Expectative strategy was finally chosen because of the rebleeding. However, these spinal aneurysms must be treated urgently because of the risk of ultra-early rebleeding.

Neurofibromatosis type 2 (NF2) is a well-studied disease. Although spinal tumors are frequent, many issues concerning their prognosis and management still have to be clarified. The authors studied the clinical impact and radiological progression of spinal tumors in patients with NF2 to determine their prognostic value. A total of 80 NF2 patients were followed in the Lille University Hospital between 1987 and 2011. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both the location and size of each tumor were reported. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation. The mean follow-up period was 8.8 years (range 1 to 24 years; SD: ±0.8), and the mean age at diagnosis was 27.2 years (range 6 to 73 years; SD: ±1.7). Among all patients, 48 harbored spinal tumors. Twenty of them were symptomatic, and 21 were operated on. Patients with spinal tumors had a lower age at diagnosis (p = 0.02), a higher number of intracranial meningiomas (p = 0.028) and schwannomas (p = 0.03), and more nonsense and frameshift mutations (p = 0.04). Spinal tumors are common in NF2, and all patients should be regularly monitored by spinal MRI. The presence of spinal tumors seems to be a factor indicating poor prognosis. Clinical and radiological monitoring of spinal tumors could lead to early treatment both when clinical symptoms are present and in case of proven radiological evolution, maintaining a favorable functional prognosis as long as possible.

Sagittal craniosynostosis (SCS) is common and easily recognized and corrected surgically. However, rare cases of SCS are more complex: these associate closure of the metopic or delayed closure of the coronal suture, uni- or bilaterally.We reviewed the available literature on atypical sagittal craniosynostosis (ASCS). We also reviewed retrospectively our series of SCS treated since 1980 and selected cases with simultaneous closure of the metopic (leptocephaly) or delayed closure of other sutures (plagiocephaly, oxycephaly, or Crouzon syndrome).ASCS is rare, representing10 % of SCS. In our series, among 447 cases of SCS followed for a mean duration of 63.7 months, we identified 22 cases of ASCS: 6 with leptocephaly, 9 with non-syndromic oxycephaly, 4 with Crouzon syndrome, and 3 with plagiocephaly. Fourteen patients required a second operation, either planned initially (severe leptocephaly) or because of brain compression. The actuarial incidence of ASCS requiring reoperation was 5.3 % of SCS at 10 years. After a mean follow-up of 113 months, morphological results in ASCS were grade 1 (no defect) in 5, grade 2 (mild defect) in 2, grade 3 (minor reoperation) in 3, and grade 4 (major reoperation) in 12; one patient had visual impairment, and two had learning difficulties.ACSC can be detected initially or occur with a delay in apparently standard SCS. Leptocephaly is a specific entity. Because of the implications on the management and risk for the patient, preoperative evaluation of patients with SCS with CT scanner and prolonged follow-up are necessary.

This report is of a rare case of glioblastoma coexisting with an arteriovenous malformation in a 65-year-old man. Multimodal magnetic resonance imaging (MRI) performed at 3T revealed a necrotic and cystic lesion in the left hemisphere; morphological and metabolic findings were consistent with an infiltrating high-grade glioma, but the presence of dark vessel-like signals on T2* and susceptibility-weighted imaging (SWI) suggested the coexistence of a vascular malformation. The arteriovenous malformation was confirmed by MR angiography and cerebral angiography. The patient was operated on, and histological examination revealed atypical cells characteristic of glioblastoma multiforme and, in the same area, arteriovenous malformation. The possible role of angiogenic factors in this case is also addressed.

The health status of adult survivors of pediatric brain tumors is poorly documented. Documenting their health risks regarding tumor recurrence or new tumor, endocrine, and cognitive sequels, as well as their social outcome, would help to define their medical needs when they become adults. Identifying risk factors of late morbidity could help to improve treatment protocols in order to reduce the burden of sequels. We decided to review retrospectively our pediatric oncology database, in order to study the oncological and clinical outcome of adult patients treated for brain tumor during childhood. We selected patients treated under the age of 18, and followed clinically after the age of 20. We studied 207 patients, aged 20.2–45 years at last control. Forty eight (23%) presented with late progression of the initial tumor, and 29 (14%) developed new tumors requiring surgery; 7 died of tumor progression. The main sequels were endocrine in 88 patients (44%), and cognitive in 86 (43%); only 36 patients (18%) had no sequel at all. The Karnofsky score was ≥80 (independent) in 73%; however, only 48% of evaluable patients were normally employed. Among the variables related to initial treatment, reoperation was the most significant factor influencing negatively outcome; however, young age at irradiation was the only significant factor predicting poor employment status. Adult patients treated for brain tumor in childhood are at significant risk of tumor progression, and many patients require prolonged oncological follow-up. Complex disabilities require the organization of efficient child-to-adult transition for these vulnerable patients.

Radiation-induced tumors (RIT) are increasingly recognized as delayed complications of brain irradiation during childhood. However, the true incidence is not established, their biology is poorly understood, and few guidelines exist regarding the long-term follow-up of irradiated children. We studied retrospectively patients irradiated for brain tumor under 18 years and followed in our institution since 1970. RIT were defined as new masses, different from the original tumor, occurring after delay in irradiated areas, and not related to phacomatosis. Among 552 irradiated patients, 42 (7.6%) developed one or more RIT, 26 months to 29 years after irradiation (mean 12.8 years). The cumulated incidence was 2.0% at 5 years and 8.9% at 10 years. Of the patients, 73.8% were adult at the time of diagnosis of RIT, and 75% were diagnosed within 18.1 years after irradiation. We identified 60 cavernomas, 26 meningiomas, 2 malignant gliomas, 1 meningosarcoma, and 6 thyroid tumors. Compared with meningiomas, cavernomas appeared earlier, in children irradiated at an older age, and with a male predominance. Although RIT were correlated with higher irradiation doses, 80.9% of these occurred at some distance from the maximum irradiation field. Twenty-five lesions were operated in 20 patients; three patients died because of progression of the RIT. A significant number of patients undergoing irradiation for brain tumor during childhood develop a RIT, often during adulthood. Our data suggest that radiation-induced cavernomas result from angiogenetic processes rather than true tumorigenesis. Protracted follow-up with MRI is warranted in children irradiated for brain tumor.

Glioblastomas are the most common CNS tumors in adults, and they remain resistant to current treatments. ErbB1 signaling is frequently altered in these tumors, which indicates that the erbB receptor family is a promising target for molecular therapy. However, data on erbB signaling in glioblastomas are still sparse. Therefore, we undertook a comprehensive analysis of erbB receptor and ligand expression profiles in a panel of nine glioblastomas that were compared to non-neoplastic cerebral tissue containing neocortex and corresponding portions of subcortical convolutional white matter and we determined the distribution patterns of erbB receptors among the main neural cell types that are present in these tumors, particularly the putative tumoral stem cell population. Using quantitative RT-PCR and western blot analysis, we showed that erbB1 signaling and erbB2 receptors exhibited highly variable deregulation profiles among tumors, ranging from under- to overexpression, while erbB3 and erbB4 were down-regulated. Immunohistochemistry revealed an important inter- and intra-tumoral heterogeneity in all four erbB expression profiles. However, each receptor exhibited a distinct repartition pattern among the GFAP-, Olig2-, NeuN- and CD133-positive populations. Interestingly, while erbB1 immunoreactivity was only detected in small subsets of CD133-positive putative tumoral stem cells, erbB3 immunoreactivity was prominent in this cell population thus suggesting that erbB3 may represent a new potential target for molecular therapy.

Thalamic tumors are uncommon, and although gross total removal (GTR) is a prospective goal, its interest is debated because the thalamus constitutes a highly functional region. The relation of choice of the surgical approach, achievability of GTR, and operative morbidity to the anatomic location of the tumor has received little attention in the medical literature.We reviewed retrospectively the cases of pediatric patients treated for thalamic tumor, with pre- and postoperative magnetic resonance imaging, and who were operated with the aim of maximal surgical removal.We reviewed 16 cases operated between 1992 and 2003. The clinical presentation was dominated by intracranial hypertension and hemiparesis. Fifteen children were operated through transcortical approaches: transfrontal in six cases, transparietal in six, and transtemporal in three. The remaining patient was operated through an infratemporal approach. All operations performed since 1998 used intraoperative neuronavigation. Complete or near-total resection was achieved in 11 cases; only subtotal resection was achieved in the remaining five cases. The most common postoperative morbidity was visual field defect. Hemiparesis was unchanged or improved in all the cases. Seven children died of tumor progression, in relation with high histological grade, and one died of acute hydrocephalus. The approach to thalamic tumors needs to be planned according to the location of critical neural structures. GTR of thalamic tumors in children bears acceptable morbidity and may even improve preoperative deficits. Surgery alone can be curative in low-grade tumors; in high-grade or infiltrating tumors, GTR is only part of the overall oncological management.

The prognosis of glioblastoma multiforme remains poor despite recent therapeutic advances. Several clinical and therapeutic factors as well as tumour characteristics have been reported as significant to survival. A more efficient determination of the prognostic factors is required to optimize individual therapeutic management. The aim of our study was to evaluate by univariate then multivariate analysis the factors that influence prognosis and particularly survival.Data of 340 patients with newly-diagnosed GBM were retrospectively analyzed. Univariate analysis of prognosis factors of survival time was performed. Factors that seemed determinant were evaluated by Kaplan-Meier survival curves. Finally, the significant factors found in univariate analysis were tested in multivariate analysis using the COX regression method.Using multivariate analysis, the following factors were found to influence survival: radiotherapy was the predominant factor followed by radical surgery, tumour location, age and chemotherapy. Patients treated with temozolomide had a markedly better survival rate than patients treated with other chemotherapies (Log-rank test P0.005). The values of GBM type (de novo or secondary), as well as repeated surgery and partial surgery (vs. simple biopsy) were suggested by univariate analysis but not confirmed by the COX regression method. After radical surgery, progression-free survival was correlated to overall survival (r = 0.87, P10e-5). CONCLUSIONS; The influence of radiotherapy on survival was greater than the influence of age, an argument supporting the proposition of radiotherapy for patients until at least age 70. In the case of recurrence, the correlation between overall survival and progression-free survival is an important factor when considering the therapeutic options. Initial radical surgery and repeated procedures dramatically influence survival. The benefit of partial surgery remains difficult to evaluate. Partial surgery could be used to decrease intracranial pressure and to minimize residual tumours in order to enable treatment by chemotherapy and radiotherapy. The value of temozolomide treatment was confirmed.