Temporal arachnoid cysts: are they congenital?

CASE REPORT: The authors report two cases of arachnoid cysts (ACs) neither detected during pregnancy nor shortly after birth when newborns underwent CT scan evaluation after birth head trauma. ACs were diagnosed at 10 months and 6 years, respectively. The first one becomes symptomatic, and the other one was incidentally found during a head trauma investigation. DISCUSSION: These cases give support for the postnatal pathogenesis for some of the assumed congenital ACs. We collected data from the literature that supports the acquired hypothesis for ACs.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

BACKGROUND: Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. CASE PRESENTATION: A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. CONCLUSION: Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Immunohistochemical expression of cyclin D1 is higher in supratentorial ependymomas and predicts relapses in gross total resection cases.

Ependymomas are tumors of the CNS. Although cyclin D1 overexpression has been related to several cancers, its prognostic value in ependymomas has not yet been fully established. We evaluated cyclin D1 expression by an immunohistochemistry analysis of 149 samples of ependymomas, including some relapses, corresponding to 121 patients. Eighty-one patients were adults, 60 were intracranial cases and 92 tumors were grade II. Gross total resection (GTR) was achieved in 62% of cases, and relapse was confirmed in 41.4% of cases. Cyclin D1 protein expression was analyzed by immunohistochemistry and scored with a labeling index (LI) calculated as the percentage of positively stained cells by intensity. We also analyzed expression of CCND1 and NOTCH1 in 33 samples of ependymoma by quantitative real-time PCR. A correlation between cyclin D1 LI score and anaplastic cases (P < 0.001), supratentorial location (P < 0.001) and age (P = 0.001) were observed. A stratified analysis demonstrated that cyclin D1 protein expression was strong in tumors with a supratentorial location, independent of the histological grade or age. Relapse was more frequent in cases with a higher cyclin D1 LI score (P = 0.046), and correlation with progression-free survival was observed in cases with GTR (P = 0.002). Only spinal canal tumor location and GTR were suggestive markers of PFS in multivarite analyses. Higher expression levels were observed in anaplastic cases for CCND1 (P = 0.002), in supratentorial cases for CCND1 (P = 0.008) and NOTCH1 (P = 0.011). There were correlations between the cyclin D1 mRNA and protein expression levels (P < 0.0001) and between CCND1 and NOTCH1 expression levels (P = 0.003). Higher cyclin D1 LI was predominant in supratentorial location and predict relapse in GTR cases. Cyclin D1 could be used as an immunohistochemical marker to guide follow-up and treatment in these cases.

Surgical approaches to IV ventricle--anatomical study.

PURPOSE: Knowledge of anatomy of the IV ventricle is basic to surgical approach of any kind of lesion in its compartment as well as for those located in its neighborhood. The purpose of this study is to demonstrate the surgical approach options for the IV ventricle, based on the step by step dissection of anatomical specimens. METHODS: Fifty formalin-fixed specimens provided were the material for this study. The dissections were performed in the microsurgical laboratory in Gainesville, Florida, USA. RESULTS: The IV ventricle in a midline sagittal cut shows a tent-shaped cavity with its roofs pointing posteriorly and the floor formed by the pons and the medulla. The superior roof is formed by the superior cerebellar peduncles laterally and the superior medullary velum on the midline. The inferior roof is formed by the tela choroidea, the velum medullary inferior, and the nodule. The floor of the IV ventricle has a rhomboid shape. The rostral two thirds are related to the pons, and the caudal one third is posterior to the medulla. The median sulcus divides the floor in symmetrical halves. The sulcus limitans runs laterally to the median sulcus, and the area between the two sulci is called the median eminence. The median eminence contains rounded prominence related to the cranial nucleus of facial, hypoglossal, and vagal nerves. The lateral recesses are extensions of the IV ventricle that opens into the cerebellopontine cistern. The cerebellomedullary fissure is a space between the cerebellum and the medulla and can be used as a surgical corridor to the IV ventricle. CONCLUSIONS: We obtained in this study a didactic dissection of the different anatomical structures, whose recognition is important for addressing the IV ventricle lesions.

Major clinical features of synostotic occipital plagiocephaly: mechanisms of cranial deformations.

PURPOSE: The clinical diagnosis of most common single-suture craniosynostosis is easily set, based on the stereotype of deformities and knowledge of the mechanisms of cranial deformations. However, synostosis of unilateral lambdoid suture, probably due to its lower incidence and similarity with other non-synostotic deformities affecting the posterior portion of the skull, makes its clinical diagnosis more difficult and imprecise. The aim of this study is to evaluate the most easily and accurate clinical characteristics to be recognized in the synostotic occipital plagiocephaly. METHODS: This study consisted of clinical evaluation of eight patients with synostotic occipital plagiocephaly, whose diagnosis was further corroborated by computed tomography. RESULTS: We identified the following: unilateral occipital flattening in eight out of eight patients (100 %), bulging of ipsilateral mastoid process in eight out of eight (100 %), "edge effect" of ipsilateral lambdoid suture in eight out of eight (100 %), inferior deviation of the ear in eight out of eight (100 %), "Dumbo" ears in eight out of eight (100 %), horizontal slant of the bimastoid line in seven out of eight (87.5 %), tilt of the head viewed from behind in seven out of eight (87.5 %), trapezoidal contour of the skull in top view in six out of eight (75 %), contralateral parietal bossing in six out of eight (75 %), and bossing of the contralateral forehead three out of eight (37.5 %). CONCLUSIONS: The most important clinical features specific to the clinical diagnosis of synostotic occipital plagiocephaly, not present in the positional posterior plagiocephaly, were bulging of the ipsilateral mastoid process, edge effect of the synostotic lambdoid suture, tilt of the head, and slant of the bimastoid line viewed from behind, inferior deviation of the ear, and contralateral parietal bossing.

High Intra-Abdominal Pressure Secondary to Obesity as a Determining Factor for Ventriculoperitoneal Shunt Malfunction

The ventriculoperitoneal shunt (VPS) is an established treatment for hydrocephalus. The functioning of the system requires a pressure difference between the cranial and abdominal cavities. The VPS can be particularly problematic in patients with increased intra-abdominal pressure (IAP). We report the case of a 16-year-old girl with VPS since she was 2 months old due to hydrocephalus secondary to myelomeningocele. The patient had been asymptomatic ever since, but she sought the emergency service with intermittent headache and vomiting. A non-enhanced brain tomography, a shunt trajectory X-ray and an abdominal ultrasound revealed no cause of system malfunction. In view of the persistent clinical picture, a revision of the shunt was performed, which revealed adequate intraoperative functioning. She returned with the same symptoms two weeks after surgery. The patient was obese (body mass index [BMI]: 48). We hypothesized intermittent valve malfunction due to increased intra-abdominal pressure. She underwent a ventriculoatrial shunt, without intercurrences. In the postoperative period, the patient presented transient tachycardia and was asymptomatic at the 6-month follow-up. Obesity should be considered an important variable for the inadequate functioning of the VPS due to increased IAP and catheter dystocia to the extraperitoneal cavity. Studies have already correlated the IAP with the BMI, which reaches between 8 mm Hg and 12 mm Hg in obese individuals. Therefore, the BMI can be considered during the selection of valve pressure in systems with non-adjustable valves to prevent insufficient drainage. The recognition of obesity as a cause of VPS malfunction is fundamental to avoid unnecessary surgeries and intermittent malfunction of the system.

A derivação ventriculoperitoneal (DVP) é um tratamento estabelecido para a hidrocefalia; contudo, algumas variáveis podem influenciar na eficácia desta modalidade. O funcionamento do sistema requer uma diferença de pressão entre as cavidades craniana e abdominal. A DVP pode ser particularmente problemática em pacientes com aumento da pressão intra-abdominal (PIA). Neste artigo, relatamos o caso de uma paciente do sexo feminino, de 16 anos, portadora de DVP desde os 2 meses de idade por hidrocefalia secundária a mielomeningocele. Desde então assintomática, procurou o pronto-socorro com queixa de cefaleia e vômitos intermitentes. Uma tomografia de crânio sem contraste, um raio X (RX) do trajeto do cateter distal, e uma ultrassonografia (USG) abdominal não evidenciaram a causa do mau funcionamento do sistema. Diante do quadro persistente, realizou-se uma revisão da derivação, que mostrou funcionamento adequado no período intraoperatório. A paciente retornou com os mesmos sintomas duas semanas após a cirurgia. A paciente era obesa (índice de massa corporal [IMC]: 48). Aventou-se possível funcionamento intermitente da válvula pelo aumento da PIA. A paciente foi submetida a uma derivação ventrículo-atrial, que foi realizada sem intercorrências. No pós-operatório, ela apresentou quadro transitório de taquicardia, e não apresentou sintomas no acompanhamento feito depois de 6 meses. A obesidade deve ser considerada uma variável importante para o funcionamento inadequado da DVP, pelo aumento da PIA e pela associação com distocia do cateter para a cavidade extraperitoneal. Estudos já correlacionaram a PIA com o IMC, que pode atingir entre8 mm Hg e 12 mm Hg em obesos. Logo, o IMC pode ser considerado na seleção da pressão da válvula em sistemas com válvulas não ajustáveis, para prevenir a drenagem insuficiente. O reconhecimento da obesidade de risco para o mau funcionamento da DVP é fundamental para evitar cirurgias desnecessárias e o mau funcionamento intermitente do sistema.

Intracranial and spinal ependymoma: series at Faculdade de Medicina, Universidade de São Paulo.

OBJECTIVE: Ependymomas are rare intracranial neuroepithelial tumors and the most common location is intramedullary. The aim was to analyze the characteristics of these tumors to determine the patients' overall survival and the likelihood of recurrence. METHOD: Data of clinical presentation, tumor location, duration of symptoms, degree of resection and complementary treatment of 34 patients with intracranial ependymoma and 31 with intramedullary ependymoma who underwent surgery in the last ten years were collected and correlated with the recurrence time and overall survival. RESULTS: There was statistically significant correlation between the degree of resection and intracranial tumor location, although it is not a hallmark of recurrence. Data analyses of intramedullary ependymoma did not show correlation with overall survival and likelihood of recurrence. CONCLUSION: The location of the intracranial tumor is connected with the degree of resection; however it is not a predictive factor to overall survival.

Estudo das alterações na expressão gênica dos ependimomas / Study of gene expression alterations in ependymomas

Ependimomas são tumores gliais raros. Podem ser encontrados em qualquer localização do sistema nervoso central e, apesar de histologia similar, parecem apresentar alterações genômicas distintas. As variáveis clínicas são intercorrelacionadas e, geralmente, incapazes de predizer o curso da doença. O objetivo do presente estudo foi analisar a expressão aumentada de genes e proteínas em ependimomas e correlacionar com dados clínicos dos pacientes. Foram estudados casos de pacientes com ependimoma submetidos à ressecção cirúrgica no Hospital das Clínicas, Universidade de São Paulo, no período entre 1996 e 2011 (33 amostras de tecido congelado para análise de expressão gênica por PCR quantitativo em tempo real e 149 amostras com tecido incluído em parafina, correspondentes a 121 casos devido a recidivas, para análise de proteína por imuno-histoquímica de tissue microarrays). As reações de imuno-histoquímica foram analisadas semiquantitativamente e graduadas com um índice de marcação calculado pelo produto da porcentagem de núcleos marcados pela intensidade de marcação. Oitenta e um casos eram adultos (média de 27,2 anos). Havia 60 casos intracranianos e 61 intramedulares, dos quais 10 eram mixopapilares, 92 grau II e 19 grau III. Ressecção completa foi possível em 62% dos casos e recidiva foi confirmada em 41,1%. Observou-se menor tempo para recidiva em crianças e tumores intracranianos, supratentoriais (p < 0,001 em ambos), histologia anaplásica e ressecções incompletas (p < 0,05 em ambos). Os seguintes genes foram selecionados em dados públicos de SAGE e literatura: ARMC3, CCND1, CHST5, DNALI1, FGFRL1, GNA13, IGF2, MSX1, NOTCH1 e RSPH3. ARMC3, RSHL3, CHST5 e DNALI1 apresentaram maiores níveis de expressão em ependimomas intramedulares (p < 0,05), e FGFRL1, NOTCH1 e CCND1 nos casos supratentoriais (p < 0,01). IGF2 apresentou maiores níveis de expressão em crianças e CHST5 em adultos (p < 0,05 em ambos). Foram observados maiores níveis de expressão de FGFRL1...

Ependymomas are rare glial cell-derived tumors. They can be found in any central nervous system localization and despite the histological similarity, they seem to display distinct genomic abnormalities. Clinical variables are intercorrelated and they are usually unable to predict the disease course. We aimed to analyze increased gene and protein expression in ependymomas and to correlate with patients' clinical data. We studied patients with ependymoma submitted to surgical resections at Hospital das Clinicas, University of São Paulo, from 1996 to 2011 (33 fresh-frozen samples for gene expression analysis by quantitative real-time PCR and 149 formalin-fixed, paraffin-embedded samples, relative to 121 patients due to relapses, for protein analysis by tissue microarray immunohistochemistry). Immunohistochemical reactions were analyzed semi-quantitatively and scored with a labeling index (LI) calculated as the product of the percentage of the positively stained nuclei by the intensity of staining. Eighty-one cases were adults (mean 27.2 years). There were 60 intracranial and 61 spinal cases, of which 10 tumors were myxopapillary, 92 were grade II and 19 were grade III. Gross total resection was achieved in 62% of cases and relapse was confirmed in 41.4% of cases. We observed a shorther time to relapse in children and supratentorial intracranial tumor localization (p<0.001 for both), anaplastic histology and incomplete resections (p<0.05 for both). The following genes were selected based on public SAGE database and literature: ARMC3, CCND1, CHST5, DNALI1, FGFRL1, GNA13, IGF2, MSX1, NOTCH1 and RSPH3. ARMC3, RSHL3, CHST5 and DNALI1 presented higher expression levels in intramedullary ependymomas (p < 0.05) and FGFRL1, NOTCH1 and CCND1 in supratentorial cases (p < 0.01). IGF2 presented higher expression levels in pediatric cases and CHST5 in adults cases (p < 0.05 in both). Higher expression levels of FGFRLI1 (p < 0.05), CCND1 and IGF2...

Intracranial and spinal ependymoma: series at Faculdade de Medicina, Universidade de São Paulo / Ependimoma craniano e de medula espinhal: casuística da Faculdade de Medicina da Universidade de São Paulo

OBJECTIVE: Ependymomas are rare intracranial neuroepithelial tumors and the most common location is intramedullary. The aim was to analyze the characteristics of these tumors to determine the patients' overall survival and the likelihood of recurrence. METHOD: Data of clinical presentation, tumor location, duration of symptoms, degree of resection and complementary treatment of 34 patients with intracranial ependymoma and 31 with intramedullary ependymoma who underwent surgery in the last ten years were collected and correlated with the recurrence time and overall survival. RESULTS: There was statistically significant correlation between the degree of resection and intracranial tumor location, although it is not a hallmark of recurrence. Data analyses of intramedullary ependymoma did not show correlation with overall survival and likelihood of recurrence. CONCLUSION: The location of the intracranial tumor is connected with the degree of resection; however it is not a predictive factor to overall survival.

OBJETIVO: Os ependimomas são tumores neuroepiteliais raros na localização intracraniana, porém um dos mais freqüentes na medula espinhal. Os autores analisaram as características destes tumores para determinar a sobrevida e probabilidade de recidiva nos pacientes. MÉTODO: Elementos da apresentação clínica, localização da lesão, duração de sintomatologia, grau de ressecção e tratamento complementar de 34 doentes com ependimoma intracraniano e 31 de medula espinhal operados nos últimos dez anos foram revisados e correlacionados com o período para a ocorrência da recidiva e a sobrevida. RESULTADOS: Houve correlação estatística apenas entre o grau da ressecção e a localização dos ependimomas intracranianos, embora, este não se tenha mostrado um marcador de recidiva. A avaliação dos dados clínicos dos pacientes com ependimoma medular não permitiu definir correlação com a sobrevida e sobre a probabilidade de recorrência. CONCLUSÃO: A localização do tumor intracraniano está relacionada ao grau de ressecção, entretanto isso não foi um fator preditivo para a sobrevida.