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This study was designed to evaluate the effects of a diet rich in fish contaminated with MeHg, mimicking the typical diet of the Amazon riverside population, in rats. Animals were randomly assigned to one of three groups with eight rats in each group: Group I—control, received commercial ration; Group II—received a diet rich in uncontaminated fish; Group III—received a diet rich in fish contaminated with MeHg. Treatment time was 12 weeks. Oxidative stress markers were evaluated, as well as the effects of this diet on DNA stability, systolic blood pressure (SBP), nitric oxide (NO) levels and histological damage in different tissues. There was a significant increase in SBP values in rats fed with MeHg-contaminated fish diet after the 10th week of the treatment. As far as oxidative stress biomarkers are concerned, no differences were observed in reduced glutathione and protein carbonyl levels, glutathione peroxidase, catalase, superoxide dismutase or δ-aminolevulinate dehydratase activities between the groups of animals receiving contaminated and uncontaminated fish diets. On the other hand, malondialdehyde levels increased significantly in rats fed with contaminated fish. NO levels were similar in all groups. DNA migration showed augmented in rats exposed to contaminated fish and histopathological analyses showed weak but significant leukocyte infiltration. Thus, we conclude that the MeHg-contaminated fish diet induced a slight lipid peroxidation and genotoxicity. However, these effects seem to be much less pronounced than when rats are exposed to aqueous solution containing CH 3 HgCl. Our findings support the contention that the chemical form of MeHg in fish or fish nutrients such as polyunsaturated fatty acids, Se or vitamin E could minimize the toxic effects of MeHg exposure in fish-eating communities.

Cancer-prone genetic disorders are responsible for brain tumors in a considerable proportion of children. Additionally, rare genetic syndromes associated to cancer development may potentially disclose genetic mechanisms related to oncogenesis.We describe two pediatric patients with encephalocraniocutaneous lipomatosis (ECCL), a very rare genetic syndrome with around 60 reported cases, which developed low-grade astrocytoma at 3 and 12 years of age.Patients with ECCL seem to be at risk of benign forms of osseous tumors such as ossifying fibromas, odontomas, and osteomas.The association between brain tumor and ECCL was previously reported only once, in a pediatric case of a mixed neuronal-glial histology. Whether ECCL may be a genetic condition of predisposing brain tumor in children strongly needs to be addressed.

Galectin-3 (Gal-3) is a glycan-binding protein highly expressed in several tumors, including brain neoplasms. This protein has been demonstrated to be correlated with adverse prognosis in some tumor types. However, the role of Gal-3 in pediatric posterior fossa tumors (PPFTs) has not yet been fully addressed. The goals of this study were to evaluate Gal-3 expression in a series of PPFTs and verify whether this expression is related to patient outcome. Gal-3 expression was analyzed by immunohistochemistry in 42 cases of surgically resected primary PPFTs. Surgeries were performed in our institution from January 2003 to December 2006. Tumor samples consisted of 21 pilocytic astrocytomas (PAs), 13 medulloblastomas, 4 ependymomas, 2 diffuse cerebellar astrocytomas, and 2 atypical teratoid/rhabdoid tumors (AT/RTs). All PAs and ependymomas strongly showed Gal-3 expression, whereas no immunostaining was observed in medulloblastomas and diffuse astrocytomas. In AT/RTs, Gal-3 expression was conspicuous but heterogeneous, being mainly observed in rhabdoid cells. Concerning the Gal-3 expressing tumors, no relationship was observed between the degree of expression and patient survival. Gal-3 was strongly expressed in reactive astrocytes, normal endothelial cells, and macrophages in the adjacent non-neoplastic brain parenchyma. Interestingly, the endothelial cells in the tumor bulk of PAs lacked Gal-3 expression. Gal-3 is differentially expressed in PPFTs, but its expression shows no correlation with patient outcome. However, the evaluation of Gal-3 is helpful in establishing a differential diagnosis among PPFTs, especially between PAs and diffuse astrocytomas, and in some circumstances between medulloblastomas and AT/RTs.

INTRODUCAO E OBJETIVOS: A Encefalite de Rasmussen (ER) e caracterizada por epilepsia intratavel, hemiparesia progressiva e atrofia hemisferica unilateral. A progressao dos sintomas geralmente ocorre em meses ou poucos anos. As drogas antiepilepticas sao usualmente ineficazes no controle da progressao da doenca e o tratamento cirurgico, com desconexao hemisferica tem sido considerado o tratamento de escolha. Neste trabalho descreveremos os achados clinicos e eletrograficos, assim como a evolucao pos-operatoria de pacientes com ER. PACIENTES E METODOS: foram incluidos todos os pacientes com ER avaliados no periodo de janeiro de 1995 a janeiro de 2008, no Centro de Cirurgia de Epilepsia de Ribeirao Preto (CIREP), sendo considerados os dados demograficos, os achados do eletrencefalograma (EEG) interictal e ictal, resultado anatomo-patologico e o seguimento clinico. RESULTADOS: Vinte e cinco pacientes foram avaliados, 13 eram do sexo feminino. A idade media de inicio da epilepsia foi de 4.4±2.0 anos. Nao houve diferencas significativas entre os pacientes com evolucao lenta ou rapida considerando-se a idade de inicio da epilepsia (p=0,79), idade da cirurgia (p=0,24), duracao da epilepsia (p=0,06) e tempo de seguimento (p=0,40). Nao houve correlacao entre a presenca de alteracoes bilaterais ou ausencia de descargas ao EEG e o seguimento pos-operatorio (p=0,06). Vinte e tres pacientes foram submetidos a cirurgia. O tempo medio de seguimento foi de 75,3 meses. Onze pacientes evoluiram com controle total das crises. Doze pacientes permaneceram com crises que consistiram de clonias faciais sutis (6 pacientes), crises tonico-clonicas hemigeneralizadas ocasionais (3 pacientes) ou crises tonico-clonicas frequentes (3 pacientes). Alteracoes cognitivas e de linguagem foram observadas em 15 e 12 pacientes apos a cirurgia, respectivamente. CONCLUSOES: este estudo retrospectivo relatou os achados clinicos, eletrograficos e a evolucao de 23 pacientes. Controle satisfatorio das crises foi obtido em 14 pacientes. Tres pacientes tiveram resposta parcial com a cirurgia e cinco pacientes mantiveram o quadro pre-operatorio. Todos os pacientes com envolvimento do hemisferio cerebral esquerdo evoluiram com disturbio de linguagem e cognitivo.

Encephalocutaneous lipomatosis (ECCL), or Haberland syndrome, is an uncommon congenital disorder with unique cutaneous, ocular and neurological features. In the present article, we describe a 3-year-old boy with ECCL who developed an extensive and recurring intraventricular low-grade glioma with atypical pathological features and elevated mitotic index. Cytogenetic analysis from tumor sample was also performed. This is the first report of a low-grade astrocytoma occurring in a child with ECCL. Whether or not the origin of the tumor is associated to the pathogenesis of the underlying syndrome is a matter for further investigation.

We searched for factors that could predispose towards persistent hydrocephalus in children with posterior fossa (PF) tumors in order to determine the need for permanent cerebrospinal fluid (CSF) diversion.The clinical records of 64 children who underwent surgery for PF tumors in the Pediatric Neurosurgery division of the Hospital of Clinics, Ribeirao Preto, Brazil, from 1990 to 2006, were retrospectively reviewed. The patients' ages ranged from 3 months to 18 years. The factors evaluated included age at surgery, severity of hydrocephalus (ventricular index), tumor location, size of the tumor, extent of tumor resection, and histology.Ventricular index, measured from the initial neuroradiological image, age at surgery, and location of the tumor were significantly associated with definitive postoperative CSF diversion (shunt or endoscopic third ventriculostomy), which was necessary for 34% of the patients.Young children with severe preoperative hydrocephalus and a midline tumor should be considered at risk when preoperative treatment decisions are made.

Extradural arachnoid cysts in the spine are uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts as communicating with the intrathecal subarachnoid space through a small defect in the dura. Excision of the cyst with obliteration of the communicating dural defect is the mainstay of treatment in symptomatic patients. Solitary extradural arachnoid cysts have been reported in several studies, but multiple extradural arachnoid cysts are very rarely reported in children. The authors report a case of multiple extradural spinal arachnoid cysts in a 14-year-old boy who presented progressive lower extremity weakness, myelopathy, and severe gait ataxia. Magnetic resonance (MR) of the spine demonstrated four extradural arachnoid cysts extending from T-1 to T-9. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cysts. Postoperatively, the patient’s motor strength and ambulation improved immediately. Multiple spinal extradural arachnoid cysts are rarely reported in the literature. Excision of the cysts at the spinal cord level leads to a favorable outcome.

Object. Two to five percent of pediatric brain tumors are located in the thalamus. The optimal management for these tumors remains unclear. The aim of this study was to determine whether clinical and neuroimaging features could guide treatment, and to what extent these features, together with histological diagnosis and treatment modalities, influenced survival. Methods. The records of 69 children who presented with a thalamic tumor between 1989 and 2003 were retrospectively reviewed. Three groups of tumors were analyzed separately: 1) unilateral thalamic tumors (54 lesions); 2) thalamopeduncular tumors (six); and 3) bilateral thalamic tumors (nine). In the patients in whom a unilateral thalamic tumor was diagnosed, 33 had an astrocytic tumor. Of the 54 patients, 32 had a low-grade and 22 had a high-grade tumor. The survival rate was significantly better for patients with the following characteristics: symptom duration longer than 2 months (p , 0.001), lesions with low-grade histological features (p = 0.003), and tumor excision greater than 90% at surgery (p = 0.04). The perioperative morbidity and mortality rates were 37 and 4%, respectively. Fifty-four percent of the patients in this group had a long-term and independent survival. The thalamopeduncular tumors were mostly pilocytic astrocytomas, which had a good prognosis following surgery. The bilateral thalamic tumors in this series were mainly low-grade astrocytic lesions, and more than half of the children attained long-term survival (mean follow-up duration 4.5 years). Conclusions. The majority of tumors arising in the thalamus are astrocytic, of which less than half are high-grade lesions. Histological evaluations should be performed in all patients in whom resection is being considered for discrete lesions. Long-term survival is possible in patients with these tumors.

The authors sought to analyze the success rate of surgery in the management of medically intractable epilepsy in children with Sturge-Weber syndrome and to determine whether the extent and timing of surgery affected seizure and developmental outcomes.The authors performed a retrospective review of 27 children who underwent surgery at their institution for medically resistant epilepsy, and they examined the outcomes with regard to epilepsy control and neuropsychological development. Seventeen children (63%) experienced onset of their epilepsy when they were younger than 1 year of age. These patients were significantly more likely to have hemiparesis (por =0.001) and status epilepticus (por = 0.001) and be developmentally delayed (por = 0.025) than children whose epilepsy started later in life. Eight patients underwent a hemispherectomy (either anatomical or functional), and complete resolution of epilepsy was noted in all. Of the 19 patients in whom a focal resection was performed, 11 (58%) became seizure free. The 10 children in whom there was residual disease were more likely to have continuing epilepsy than the nine whose lesions were completely excised (por = 0.05). Seventeen children exhibited improvement in their developmental status following surgery. This improvement was significantly affected by completeness of resection (por = 0.05) and age at surgery (por = 0.009). Seizure freedom per se was not affected by the timing of surgery.Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.

Hydrocephalus is a complex disease that affects cerebrospinal fluid (CSF) dynamics and is very common in children. To this date, CSF shunting is still the standard treatment for childhood hydrocephalus, but, nevertheless, the effects of such an operation on the developing brain are widely unknown. To help overcome this, experimental models of CSF shunts are surely very useful tools. The objective of this study was to describe a feasible and reliable technique of an adapted ventricular-subcutaneous shunt for the treatment of kaolin-induced hydrocephalus in young rats. We developed a ventricular-subcutaneous shunt (VSCS) technique which was used in 31 Wistar young rats with kaolin-induced hydrocephalus. Hydrocephalus was induced at 7 days of age, and shunt implantation was performed 7 days later. Our technique used a 0.7-mm gauge polypropylene catheter tunneled to a subcutaneous pocket created over the animal’s back and inserted into the right lateral ventricle. All animals were sacrificed 14 days after shunt insertion. Twenty-four rats survived and remained well until the study was ended. No major complications were seen. Their weight gain went back to normal. They all underwent ambulatory behavioral testing prior and after VSCS, which showed improvement in their motor skills. We have also obtained magnetic resonance (MR) scans of 16 pups confirming reduction of ventricular size after shunting and indicating effective treatment. Histopathological analysis of brain samples before and after shunting showed reversion of ependymal and corpus callosum disruption, as well as fewer reactive astrocytes in shunted animals. An experimental CSF shunt technique was devised. Excessive CSF of hydrocephalic rats is diverted into the subcutaneous space where it can be resorbed. This technique has a low complication rate and is effective. It might be applied to various types of experimental studies involving induction and treatment of hydrocephalus.

Up to 20% of patients with pilocytic astrocytoma (PA) experience a poor outcome. BRAF alterations and Fibroblast growth factor receptor 1 (FGFR1) point mutations are key molecular alterations in Pas, but their clinical implications are not established. We aimed to determine the frequency and prognostic role of these alterations in a cohort of 69 patients with PAs. We assessed KIAA1549:BRAF fusion by fluorescence in situ hybridization and BRAF (exon 15) mutations by capillary sequencing. In addition, FGFR1 expression was analyzed using immunohistochemistry, and this was compared with gene amplification and hotspot mutations (exons 12 and 14) assessed by fluorescence in situ hybridization and capillary sequencing. KIAA1549:BRAF fusion was identified in almost 60% of cases. Two tumors harbored mutated BRAF. Despite high FGFR1 expression overall, no cases had FGFR1 amplifications. Three cases harbored a FGFR1 p.K656E point mutation. No correlation was observed between BRAF and FGFR1 alterations. The cases were predominantly pediatric (87%), and no statistical differences were observed in molecular alterations-related patient ages. In summary, we confirmed the high frequency of KIAA1549:BRAF fusion in PAs and its association with a better outcome. Oncogenic mutations of FGFR1, although rare, occurred in a subset of patients with worse outcome. These molecular alterations may constitute alternative targets for novel clinical approaches, when radical surgical resection is unachievable., This study was partially supported by CNPq/Universal (475358/2011-2), and FAPESP (2012/19590-0) grants to RMR and to the NIH- P30CA046934 (CCSG Molecular Pathology/Cytogenetics) to MVG and DLA

ObjectivesPituitary stem cells play a role in the oncogenesis of human adamantinomatous craniopharyngiomas (aCPs). We hypothesized that crosstalk between the Wnt/β-catenin and Sonic Hedgehog (SHH) pathways, both of which are important in normal pituitary development, would contribute to the pathogenesis of aCPs.DesignTo explore the mRNA and protein expression of components of the SHH signaling pathway in aCPs and their relationship with the identification of CTNNB1/β-catenin mutations and patients outcomes.Patients and methodsIn 18 aCP samples, CTNNB1 was sequenced, and the mRNA expression levels of SHH pathway members (SHH, PTCH1, SMO, GLI1, GLI2, GLI3, and SUFU) and SMO, GLI1, GLI3, SUFU, β-catenin, and Ki67 proteins were evaluated by quantitative real-time PCR and immunohistochemistry respectively. Anterior normal pituitaries were used as controls. Associations between molecular findings and clinical data were analyzed.ResultsThe aCPs presented higher mRNA expression of SHH (+400-fold change (FC); PGLI1 (+102-FC; PGLI3 (+5.1-FC; PSMO and SUFU mRNA expression (PP=0.02 respectively). CTNNB1/β-catenin mutations were found in 47% of the samples. aCPs with identified mutations presented with higher mRNA expression of SMO and GLI1 (+4.3-FC; P=0.02 and +10.2-FC; P=0.03 respectively). SMO, GLI1, GLI3, and SUFU staining was found in 85, 67, 93, and 64% of the samples respectively. Strong GLI1 and GLI3 staining was detected in palisade cells, which also labeled Ki67, a marker of cell proliferation.ConclusionsThe upregulation of SHH signaling occurs in aCPs. Thus, activation of Wnt/β-catenin and SHH pathways, both of which are important in pituitary embryogenesis, appears to contribute to the pathogenesis of aCP.

BACKGROUND/OBJECTIVES Pilocytic astrocytomas (PAs) are the most frequent astrocytomas in children and adolescents. Methilthioadenosine phosphorylase(MTAP) is a tumor-suppressor gene, the loss of expression of which is associated with a poor prognosis and better response to specific chemotherapy in leukemia and non-small-cell lung cancer. The expression of MTAP in brain tumors remains largely unknown and its biological role in PA is still unexplored. Our aims were to describe the immunohistochemical MTAP expression in a series of PAs and relate it to the clinicopathological features of the patients. METHODS We assessed MTAP expression on immunohistochemistry in 69 pediatric and adult patients with PA in a tissue microarray platform. RESULTS Retained expression of MTAP was seen in >85% of the tumors compared to in the nonneoplastic adjacent tissue. Only 3 supratentorial tumors showed a complete loss of MTAP expression. No significant association with clinicopathological features or overall survival of the patients was found. CONCLUSIONS MTAP expression is retained in PAs and is not an outcome predictor for these tumors. Nevertheless, a subset of patients with PAs exhibiting a loss of MTAP could potentially benefit from treatment with specific chemotherapy, especially when lesions are recurrent or surgical resection is not recommended., This study was partially supported by the Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (2012/19590-0) and an HCB program of financial support (Programa de Apoio e Incentivo a Pesquisa).

This study aims to evaluate the impact of gross total resection of giant supratentorial brain tumors (GSBT) on survival and neurological outcome in a consecutive single-center pediatric series. Clinical data of 23 patients under 18 years of age operated with GSBT (≥5 cm in diameter) were reviewed to determine epidemiological aspects, clinical presentation, associated factors, histopathological features, and outcome. Volumetric measurements were performed on magnetic resonance imaging or computed tomography scans obtained at the time of the initial surgical procedure. The group included 23 patients (mean age 4.5 years). Signs and symptoms of raised intracranial pressure were present in 19 patients (82.6 %). The most frequent tumor location was the parietal lobe in 19 patients (82.6 %), and the mean tumor volume was 208 cm3. Gross total or radical resection was achieved in all patients. Histopathological analysis revealed malignant brain tumors in 18 cases (78.2 %). The most common neoplasm was choroid plexus carcinoma in seven (30.4 %). Mean intraoperative blood transfusion volume was 51.2 ml/kg. Chemotherapy and/or radiotherapy were performed as adjuvant treatment in 16 patients (69.5 %). Mean length of follow-up was 36.7 months. Tumor malignancy grade significantly correlated with recurrence of the disease (P = 0.03) and death (P = 0.01), as opposed to tumor location, size, and extension to the ventricles. Our clinical experience suggests that tumor mass reduction by en bloc surgery seems to be an effective approach in pediatric patients with GSBT, relieving symptoms related to raised intracranial pressure and providing a better response to adjuvant treatment.

Neonatal brain abscesses are very rare and their clinical presentation is specific for this age group. They usually occur as a complication of bacterial meningitis or septicemia. They are most often caused by gram-negative organisms, and mortality and morbidity are still significant in this particular group of patients in spite of antibiotics and modern radiological tools.We report an unusual case of a multiloculated brain abscess in a term neonate caused by Staphylococcus aureus. The abscess developed in the absence of trauma, prior surgery, cyanotic heart disease, mother's disease, or immune defect. The onset of infection in this case was not clear and image features mimicked a brain tumor in the initial evaluation. The infant was successfully treated by primary surgical excision of the lesion and a 6-week total course of intravenous antibiotics.The interest of this case lies in the rarity of the causative organism and the atypical features of clinical and neurological images in a term neonate.

Aplasia cutis congenita (ACC) is a rare disease of unknown etiology, involving any site of the body. The scalp is the most frequent location, followed by the forearms, knees, both sides of the trunk, and neck, in decreasing order of frequency. Superficial lesions may heal spontaneously and seldom result in morbidity or mortality. However, in patients with large scalp and skull defects, there are risks of infection and bleeding. Conservative treatment has been described and advocated, but some authors have highlighted the disadvantages of this treatment modality. On the other hand, several authors claim that aggressive surgical treatment has an important role for large defects.In this paper, we report three newborns with ACC of the scalp, two large defects and a medium one, respectively, treated conservatively and surgically.The management of ACC of the scalp is still controversial. Our series suggests that conservative treatment should be performed for initial management in newborns.

Object. The authors of this retrospective review and analysis of the literature cover an institutional series of neurenteric cysts of the central nervous system in children treated in the magnetic resonance imaging era during a 14-year period. Methods. Sixteen patients 20 days to 14 years of age are described. The most frequent signs and symptoms at presentation were acute spinal cord compression (11 patients), paresis of a cranial nerve (two patients), meningitis or infection (two patients), and intracranial hypertension (one patient). The locations of the cysts were as follows: in the spinal canal in 12 patients (75%), the clivus in two (12.5%), the cavernous sinus in one (6%), and the craniocervical junction in one (6%). The most common location was the ventral aspect of the spinal canal (seven patients). Associated spinal deformities were found in five patients. All patients underwent surgery, with a posterior approach used in all of the spinal cases. Total resection was achieved in 12 of the 16 cases and partial resection in four. Of the four patients who underwent subtotal resection, the cyst recurred in three, requiring further surgery. Conclusions. Neurenteric cysts are uncommon congenital anomalies that can present acutely in the pediatric population. Total removal is usually possible and is associated with a good prognosis.

Ependymoma is a central nervous system neoplasm that is often managed with surgery and radiotherapy. The benefits of chemotherapy in treating this tumor remain poorly defined. The use of a platinum-based chemotherapy as a neoadjuvant treatment to permit complete surgical resection of a supratentorial anaplastic ependymoma recurring for the third time is described. This paper also discusses the possible use of chemotherapy as a strategy that may allow tumor shrinkage and ease tumor resection in giant recurring ependymomas.

INTRODUÇÃO: O presente trabalho analisou 150 casos consecutivos de tratamento da hidrocefalia com DVP no Hospital das Clínicas de Ribeirão Preto entre março de 1997 e julho de 2000. OBJETIVO: Traçar as principais características dos pacientes e dos procedimentos, com ênfase nas etiologias, diagnóstico, complicações, seqüelas e fatores associados. MÉTODOS: Prontuários médicos como fonte para quantificar as variáveis selecionadas. RESULTADOS: As etiologias congênitas e adquiridas tiveram a mesma incidência, destacando-se a mielomeningocele no primeiro grupo e a prematuridade e a meningite no segundo. As principais complicações foram o mal funcionamento da válvula (33%) e a infecção (15%). Incluídas as cirurgias devido às complicações, houve 2,5 procedimentos por paciente em média. No último retorno, 40% dos pacientes apresentavam algum grau de retardo do desenvolvimento neuro-psico-motor (RDNPM). As principais etiologias relacionadas a RDNPM foram prematuridade, meningite e malformações complexas. Discussão: O trabalho serviu como ferramenta para ajudar a caracterizar a história natural da hidrocefalia e de seu tratamento em nosso meio, fornecendo base para uma melhor compreensão da mesma e para comparação com a literatura e com outros serviços. A taxa de RDNPM está condizente com a literatura. A taxa de infecção está mais elevada, podendo haver relação com o fato de ser este um hospital-escola. Maior tempo de seguimento seria necessário para comparação da incidência de complicações mecânicas. INTRODUCTION/OBJECTIVES: This study has analyzed 150 consecutive cases of treatment of hydrocephalus by ventriculoperitoneal shunt in the Faculty of Medicine of Ribeirão Preto's Hospital between May 1997 and July 2000, in order to stablish the main characteristics of the patients and proceedings , in particular the etiologies, diagnosis, complications, final outcome and associated factors. METHODS: Medica records as a source for assesment of the selected variables. RESULTS: The congenital and the acquired etiologies had the same incidence, with the complexes malformations and the complications of prematurity and meningitis with distinction in each group respectively. The main complications were malfunction of the valve (33%) and infection (15%). Including the operations caused by complications, there were 2,5 surgical proceedings to each patient, on average. In the final examination, 40% of the patients had some level of neurological deficit. The main etiologies related to this deficits were prematurity , meningitis and the complexes malformations. DISCUSSION: This report has contributed to characterize the evolution and treatment of hydrocephalus in our environment, producing basis to make comparisons with the literature and with other services. The rate of neurological deficit agrees with the literature. The rate of infection is higher, maybe because it is a Universitary Hospital. A longer period of observation would be necessary to compare the incidences of mechanical complications.

Teratomas are very rare intracranial tumors and cytogenetic information on this group remains rare. We report a case of a mature teratoma with abnormal +21 trisomy in tumor karyotype ocurring in a non-Down syndrome(DS) infant. Additionally, the evidence for the contribution of chromosome 21 trisomy in this neoplasia are briefly reviewed. The 6-month-old male baby presented with a posterior fossa tumor. Histological evaluation of tumor specimen showed a mature teratoma composed of fully differentiated ectodermal, mesodermal and endodermal components. Although somatic karyotyping of the index case was normal, composite tumor karyotype depicted 47,XY,+21[6]/46,XY[6]. Besides previous reports of children with DS and intracranial teratomas, this is the first report to describe the occurrence of an isolated chromosome 21 trisomy within the tumor of a non-DS child. The participation of chromosome 21 in this rare pediatric tumor, either somatic or restricted to tumor specimen,may deserve special interest and further investigation.

Brain tumors are a frequent cause of epilepsy in the pediatric population. The last International League Against Epilepsy (ILAE) classification of focal cortical dysplasias (FCDs) includes a subgroup consisting of tumors with surrounding dysplastic abnormalities (FCD type IIIb). Although its pathogenesis is still unclear, it has several clinical and therapeutic (surgical) implications. A number of benign tumors (such as dysembryoplastic neuroepithelial tumors and gangliogliomas) frequently present with medically refractory epilepsy associated with cortical dysplasia. In such cases, planning of surgical resection needs to take into consideration not only the tumor but also the whole area of epileptogenicity. The use of intraoperative electrocorticography recordings is reported to result in better postoperative outcomes, since they help delineate the abnormal cerebral cortex that needs to be resected to provide seizure freedom to patients. Clinical, radiological, and pathological features are also discussed herein.

Highly active antiretroviral therapy (HAART) has resulted in dramatic decreases in morbidity and improved survival for adults and children with HIV infection [1–3]. However, even as the risk of AIDS-defining illness has decreased, other complications have become more significant causes of morbidity and mortality in long-term survivors. Liver failure and cirrhosis have emerged as leading non-AIDS causes of illness and death in HIV-infected adults, especially in those with hepatitis B (HBV) or C (HCV) co-infection [4]. While co-infections, hepatotoxic antiretroviral medications (ARVs) and other drugs, severe immunosuppression, alcohol use and other traditional (non-HIV-specific) risk factors for hepatic dysfunction have been implicated, hepatic fibrosis and dysfunction also occur in adults with longstanding HIV infection and no other specific risk factors [5–6]. Hepatitis and liver dysfunction are recognized complications of perinatal HIV infection, but the prevalence of clinically recognized disease appears to be much lower than that in adults [7–9]. However, as these children now survive into adulthood and face lifelong HIV infection and treatment, hepatic dysfunction may emerge as an important cause of morbidity and even mortality. The standard for assessing presence and degree of liver inflammation, fibrosis and cirrhosis is examination of a liver biopsy, but this diagnostic approach is highly invasive and unsuitable for potential hepatic fibrosis screening. The aspartate aminotransferase [AST]-to-platelet ratio index (APRI) is a non-invasive measure of significant hepatic fibrosis that has been used in studies of HIV-mono-infected and HIV/HCV co-infected adults [10–18]. An APRI >1.5 has been validated as evidence of significant liver fibrosis in HIV-HCV co-infected adults [12–14]. In studies of HIV-uninfected children with chronic viral hepatitis and other liver conditions, elevated APRI has been shown to be associated with liver fibrosis - including validation with liver biopsy findings - especially with more advanced stages of liver fibrosis [19–22]. The best APRI cutoff for children has not been established; the adult APRI cutoff of 1.5 appears to be highly specific but relatively insensitive for detection of liver fibrosis in children [19]. APRI has not been previously studied in HIV-infected children. The purpose of this study is to estimate the rate of elevated APRI, as a marker of potential liver fibrosis, in a cohort of perinatally HIV- infected Latin American children.

Medical School of Ribeirao Preto, University of Sao Paulo, Ribeirao Preto SP, Brazil: Department of Internal Medicine, Division of Radiology; Department of Surgery, Division of Pediatric Neurosurgery. Intracranial pial arteriovenous fistulas (AVFs) are an extremely rare cerebrovascular disease, accounting for 1.6% of all intracranial vascular malformations. It has been recognized as a pathological entity distinct from the arteriovenous malformations (AVMs), due to the direct connection between the feeder artery and the draining vein, without an intervening true nidus. Pial AVFs differ from dural AVFs in that their arterial supply derive from pial and cortical arteries and they are not involved by the leaflets of the dura-mater. They are in their majority considered to be congenital, but they may also occur sporadically or in association with congenital hereditary vasculopathies. The pial AVFs have a significant morbid-mortality secondary to its rupture when not treated. We report a case of intracranial pial fistula in a 2 years-old patient presenting seizures since the neonatal period, emphasizing the angiographic findings and the results of the endovascular treatment.

Univ Sao Paulo, Fac Philosophy Sci & Letters, Div Pediat Oncol, Dept Pediat, Sao Paulo, Brazil

A 22-year-old woman was examined for a complaint of bilateral progressive enophthalmos that had begun after the cerebrospinal fluid shunting procedure 9 years ago. Photographs and CT scans taken before surgery proved that the position of her eyes was normal before surgery. The enophthalmos was so severe that it induced a poor eyelid-globe apposition with trichiasis and superficial keratopathy. CT of the orbits showed that both orbital roofs were arched and displaced toward the anterior cranial fossa. The placement of porous polyethylene sheets on the orbital roofs through a coronal approach corrected the eye position. A literature review indicated that cerebrospinal shuntings are plagued by a variety of complications including bone changes and craniosynostosis. We believe that enophthalmos associated with cerebrospinal fluid shunting results from a rare acquired bony orbital anomaly.

John Cunningham (JC) viral DNA sequence has seldom been reported in patients with brain tumors such as high grade gliomas and medulloblastomas, pointing to a role in the etiopathogenesis of such tumors. We present a unique clinical case of an HIV-positive pediatric patient with multifocal leukoencephalopathy and confirmed JC virus (JCV) infection that developed a giant-cell glioblastoma. Experimental data with infected primates has previously hypothesized an association of human giant-cell glioblastoma with JCV or progressive multifocal leukoencephalopathy, though such association has not been documented in the literature for humans. Future studies with larger cohorts and molecular pathological analyses are still needed to corroborate the role of the widely spread human neurotropic virus in early transformation and in the development of brain tumors with different histology in the setting of HIV-related severe immunosuppression.

Myosin Va functions as a processive, actin-based motor molecule highly enriched in the nervous system, which transports and/or tethers organelles, vesicles, and mRNA and protein translation machinery. Mutation of myosin Va leads to Griscelli disease that is associated with severe neurological deficits and a short life span. Despite playing a critical role in development, the expression of myosin Va in the central nervous system throughout the human life span has not been reported. To address this issue, the cerebellar expression of myosin Va from newborns to elderly humans was studied by immunohistochemistry using an affinity-purified anti-myosin Va antibody. Myosin Va was expressed at all ages from the 10th postnatal day to the 98th year of life, in molecular, Purkinje and granular cerebellar layers. Cerebellar myosin Va expression did not differ essentially in localization or intensity from childhood to old age, except during the postnatal developmental period. Structures resembling granules and climbing fibers in Purkinje cells were deeply stained. In dentate neurons, long processes were deeply stained by anti-myosin Va, as were punctate nuclear structures. During the first postnatal year, myosin Va was differentially expressed in the external granular layer (EGL). In the EGL, proliferating prospective granule cells were not stained by anti-myosin Va antibody. In contrast, premigratory granule cells in the EGL stained moderately. Granule cells exhibiting a migratory profile in the molecular layer were also moderately stained. In conclusion, neuronal myosin Va is developmentally regulated, and appears to be required for cerebellar function from early postnatal life to senescence.

Mowat-Wilson syndrome (MWS) is a rare genetic condition where variable and multiple congenital anomalies including Hirschsprung's disease, intellectual disability, and prominent facial features are present. At molecular level, MWS is characterized by many different described mutations in the zinc finger E-box protein 2 (ZEB2) gene, ultimately leading to loss of gene function. This report is the first to describe the association of MWS with two different asynchronous malignant brain tumors (medulloblastoma and glioblastoma) occurring in a child.

Objectives: To determine the clinical presentation and treatment outcome of pediatric intracranial cavernous malformation (CM) in a single-centered institution. Methods: Clinical data review of 30 patients under 18 years-old who had undergone surgery for cavernous malformation from January 1993 to December 2011. Results: The Study Group included 18 males and 12 females (mean age: 8.7 years-old). Symptoms at presentation were seizures (16/30, 53.3%), headache (15/30, 50.0%), and focal neurological deficits (11/30, 36.6%). Multiple cavernous malformations were found in 5/30 (16.6%). According to location, patients were classified in groups: (G1) brain-steam in 5/30 (16.6%), (G2) cerebellum in 2/30 (6.6%), (G3) supratentorial associated with seizures in 16/30 (53.3%), and (G4) supratentorial without seizures in 7/30 (23.3%). Surgical resection was performed in 26 out of 30 (86.6%) patients. The mean follow-up period was 4.1 years. Of 15 children followed-up with preoperative seizures, all were rendered seizure-free after surgery. Conclusions: For symptomatic solitary cavernous malformation, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. For multiple cavernous malformation or asymptomatic patients, the treatment modalities must be cautiously considered. Objetivos: Determinar a apresentação clínica e o acompanhamento do tratamento em crianças com angioma cavernoso intracraniano numa única instituição. Métodos: Revisão de dados clínicos de 30 pacientes menores de 18 anos com que passaram por uma cirurgia de angioma cavernoso intracraniano entre janeiro de 1993 a dezembro de 2011. Resultados: O grupo de estudo incluiu 18 sujeitos masculinos e 12 femininos (idade média: 8,7 anos). Os sintomas iniciais eram convulsões (16/30, 53,3%), cefaleia (15/30, 50,0%) e déficits neurológicos focais (11/30, 36,6%). Havia angiomas cavernosos intracranianos múltiplos em 5 de 30 (16.6%). A classificação foi feita em grupos de acordo com a localização: (G1) tronco cerebral em 5/30 (16,6%); (G2) cerebelo em 2/30 (6,6%); (G3) supratentoriais associados a convulsões em 16/30 (53,3%) e (G4) supratentoriais sem convulsões em 7/30 (23,3%). Ressecção cirúrgica foi realizada em 26 de 30 (86,6%) pacientes, com seguimento médio de 4,1 anos. De 15 crianças com convulsões pré-operatórias, todas ficaram livres das crises após a cirurgia. Conclusões: Para angioma cavernoso intracraniano solitário e sintomático, o tratamento de escolha é excisão microcirúrgica total precedida de avaliação funcional e anatômica meticulosa. Para angiomas cavernosos intracranianos múltiplos ou pacientes assintomáticos, as modalidades terapêuticas devem ser consideradas cautelosamente.

ObjectiveNeuroendoscopic surgery in children has particular features and is associated with different success rates (SR). The aim of this study was to identify putative factors that could influence the outcome in pediatric patients.MethodsClinical data of 177 patients under 18 years of age submitted to 200 consecutive neuroendoscopic procedures from January 2000 to January 2010 were reviewed.ResultsThe overall success rate was 77%. Out of the patients with successful outcomes, 46% were under six months, 68% were between six months and one year of age, and 85% older than one year. Neuroendoscopic techniques provide very good results for a wide number of indications in children. Tumor-related cerebrospinal fluid (CSF) circulation problems and aqueductal stenosis seem to be particularly well suited to neuroendoscopic treatment regardless of the patient's age.ConclusionPatients' age and etiology of hydrocephalus were associated with a different outcome. In all cases, surgical experience is extremely important to reduce complications. ObjetivoA cirurgia neuroendoscópica em crianças apresenta particularidades e está associada a diferentes taxas de sucesso (TS). O objetivo deste estudo consistiu em identificar fatores que pudessem influir no resultado do tratamento em pacientes pediátricos.MétodosDados clínicos de 177 pacientes com idade inferior a 18 anos submetidos a 200 procedimentos neuroendoscópicos consecutivos entre janeiro de 2000 e janeiro de 2010 foram revisados.ResultadosA taxa de sucesso global foi de 77%. Os pacientes com idade inferior a seis meses apresentaram taxa de sucesso de 46%; pacientes entre seis meses e um ano de vida obtiveram êxito em 68% dos casos; dentre os maiores de um ano, 85% dos procedimentos foram bem-sucedidos. Técnicas neuroendoscópicas proporcionam muito bons resultados para uma grande variedade de indicações em crianças. Independentemente da faixa etária, o tratamento endoscópico apresenta-se particularmente adequado para problemas da circulação liquórica relacionados a tumores e à estenose aquedutal.ConclusãoA faixa etária dos pacientes e a etiologia da hidrocefalia estão associadas a diferentes resultados. Em todos os casos, experiência neurocirúrgica é extremamente importante para a redução das complicações.

Epidermoid cysts of the cerebellopontine angle (CPA) can be a surgical challenge for the pediatric neurosurgeon. Ideally, total removal must be achieved; however, occasional adhesions of these tumors to vital neurovascular structures and extension far beyond the midline may preclude their total removal. The aims of this article are to present an alternative surgical approach to these lesions and to provide the rationale for this technique.A 16-year-old boy was admitted to our pediatric neurosurgery department with a 1-year history of nonspecific headaches. His neurological examination showed right-sided dysmetria and gait ataxia. Magnetic resonance scans showed a space-occupying lesion on the right CPA with low intensity on T(1)-weighted images and high intensity on T(2)-weighted images.Craniotomy for tumor excision via pre- and subtemporal transtentorial approach was performed disclosing a 3.5 × 3 × 2.8-cm(3) well-encapsulated tumor, which was confirmed to be an epidermoid cyst. The postoperative course was uneventful.A combined pre- and subtemporal approach utilizes a wide opening of the tentorium and the option of supratentorial retraction of the cerebellum to provide an excellent angle of approach to CPA lesions involving the anterolateral aspect of the brain stem in children.

Ionizing radiation is the most recognized risk factor for meningioma in pediatric long-term cancer survivors. Information in this rare setting is exceptional. We report the clinical and cytogenetic findings in a radiation-induced atypical meningioma following treatment for desmoplastic medulloblastoma in a child. This is the second study to describe the cytogenetic aspects on radiation-induced meningiomas in children. Chromosome banding analysis revealed a 46, XX, t(1;3)(p22;q12), del(1)(p?)[8]/46, XX[12]. Loss of chromosome 1p as a consequence of irradiation has been proposed to be more important in the development of secondary meningiomas in adults. Deletions in the short arm of chromosome 1 also appear to be a shared feature in both pediatric cases so far analyzed.

The goal of this study was to determine the epidemiology, clinical presentation, associated factors, pathological features, and treatment outcome of pediatric meningiomas in a single-center institution. Clinical data of 15 patients under 18 years of age operated on for meningiomas from January 1994 to December 2010 were reviewed. The study group included nine males and six females (mean age of 13 years at surgery). The most common symptoms at presentation were headaches in 6 out of 15 (40 %), raised intracranial pressure in 3 out of 15 (20 %), and seizures in 3 out of 15 (20 %). Sole operated tumors were found in 12 out of 15 (80 %), whose location is as follows: parasagittal in 4 out of 12 (33.3 %), 2 in the convexity (16.6 %), 2 at the skull base (16.6 %), and 4 in other sites (33.3 %). Six children presented with radiation-induced (RT) meningiomas and five had evidence of neurofibromatosis type 2 (NF2). Three patients had multiple meningiomas (all of them had NF2). Simpson’s grade I excision was achieved in 12 out of 15 (80 %). On histopathology, 11 out of 15 (73.3 %) were grade I and 4 out of 15 (26.6 %) were grade II (all of them atypical). Five tumors (33.3 %) recurred, four of which had RT or NF2. During the mean follow-up period of 5 years, 12 out of 15 (80 %) had a good outcome (GOS = 5). Childhood meningiomas are uncommon lesions with a slight male predominance. Absence of large series with long follow-up precludes any definite conclusions on the clinical course and outcome of these tumors. Associated factors (such as RT and NF2), location, and extent of excision appear to be more important than histopathological grade in predicting outcome.

A single-center experience with pediatric patients who underwent surgery for intractable rolandic epilepsy was reviewed with the aim of identifying putative factors that could influence postoperative seizure outcome in this population.Clinical data of 48 patients under 18 years of age with diagnosis of intractable rolandic epilepsy who underwent surgery from January 1996 to September 2009 were reviewed.Patients' mean age at surgery was 9.9 ± 5.3 years; mean age at epilepsy onset was 3.9 years; mean seizure duration prior to surgery was 6 years; and mean follow-up was 5.1 years. The most frequent etiologies were cortical dysplasia, astrogliosis, tumors, tuberous sclerosis complex, and Sturge-Weber syndrome, which were observed in 20/48 (41.6%), 10/48 (20.8%), 10/48 (20.8%), 5/48 (10.4%), and 3/48 (6.2%) of the patients, respectively. After surgery, 20 patients (41.6%) showed neurological deficits, which in turn recovered within no longer than 6 months after surgery. Seizure outcome was classified as Engel class I in 29 (60.4%), Engel class II in 10 (20.8%), and Engel class III in 9 (18.8%) of the patients. The factors significantly related with seizure outcome were histological features (tumor versus non-tumor cases, p = 0.04) and lesion site (focal lesions versus non-focal lesions, p = 0.04).Tailored resection of rolandic cortex for intractable epilepsy can be safely performed in children. Accurate mapping of both functional cortex and epileptogenic areas may lead to improved seizure outcome. Tumor as well as focal lesions in hand and face motor areas are associated with good seizure outcome.

OBJECTIVE: The aim of this study was to review the results of surgery for pediatric patients with Cushing's disease who were less than 18 years old and underwent transsphenoidal surgery in a specialized center during a 25-year period. SUBJECTS AND METHODS: Retrospective study, in which the medical records, histology and pituitary imaging of 15 consecutive pediatric patients with Cushing's disease (mean age: 13 years) were evaluated by the same team of endocrinologists and a neurosurgeon from 1982 to 2006. Patients were considered cured when there was clinical adrenal insufficiency and serum cortisol levels were below 1. 8 µg/dL or 50 nmol/L after one, two, three, or seven days following surgery; they therefore required cortisone replacement therapy. Follow-up was for a median time of 11.5 years (range: 2 to 25 years). RESULTS: Clinical and biochemical cure was achieved in 9/15 patients (60%) exclusively after transsphenoidal surgery. Hypopituitarism was observed in four patients; growth hormone deficiency, in two; permanent diabetes insipidus, in one case. CONCLUSIONS: Cushing's disease is rare in children and adolescents. Transsphenoidal surgery is an effective and safe treatment in most of these patients. Plasma cortisol level < 1. 8 µg/dL following surgery is the treatment goal and is a good predictive factor for long-term cure of Cushing's disease. OBJETIVO: O objetivo deste estudo foi avaliar os resultados cirúrgicos em pacientes pediátricos com doença de Cushing com idade inferior a 18 anos, submetidos à cirurgia transfenoidal num centro especializado, durante um período de acompanhamento de 25 anos. SUJEITOS E MÉTODOS: Estudo retrospectivo dos prontuários médicos de 15 pacientes pediátricos com doença de Cushing (idade média de 13 anos), sendo avaliados aspectos clínicos, laboratoriais, histológicos e radiológicos. Todos os pacientes foram avaliados pela mesma equipe de endocrinologistas e operados por um mesmo neurocirurgião, entre 1982 e 2006. O tempo médio de seguimento foi 11,5 anos (2 a 25 anos). Os pacientes foram considerados curados quando houve insuficiência adrenal e níveis de cortisol plasmático inferiores a 1,8 µg/dL ou 50 nmol/L no pós-operatório um, dois, três ou sete dias após a cirurgia; estes pacientes necessitaram de reposição de corticosteroide. RESULTADOS: Cura clínica e bioquímica foi alcançada em 9/15 pacientes (60%) após a cirurgia transfenoidal. Hipopituitarismo foi observado em quatro pacientes; déficit de hormônio de crescimento, em dois; diabetes insípido permanente, em um. CONCLUSÕES: A doença de Cushing é rara na infância e na adolescência. A cirurgia transfenoidal é um tratamento efetivo e seguro para a maioria dos pacientes. Uma concentração de cortisol plasmático < 1,8 µg/dL nos primeiros dias pós-cirurgia transfenoidal é o objetivo do tratamento e um fator preditivo tardio para a cura da doença de Cushing.

This study described a 23-year experience in the treatment of children with pilocytic astrocytomas (piloA) with the aim of identifying putative clinical, histopathological, and/or immunohistochemical features that could be related to the outcome of these patients.Clinical data of 31 patients under 18 years of age with piloA were obtained from 1984 to 2006.The mean age at the time of surgery was 7.8 +/- 4.2 years (1 to 17 years), and the mean follow-up was 5.7 +/- 5.4 years (1 to 20 years). The most common site of tumor formation was the cerebellum (17), followed by brainstem (4), optic chiasmatic hypothalamic region (4), cerebral hemisphere (3), cervical spinal cord (2), and optic nerve (1). Gross total resection (GTR) was achieved in 23 (74.1%), mainly in those with tumors located in the cerebellum and cerebral hemispheres (P = 0.02). The global mortality rate was 6.4%. Nine patients were reoperated. Rosenthal fibers, eosinophilic granular bodies, microvascular proliferation, and lymphocytic infiltration were observed in most cases. The mean Ki-67LI was 4.4 +/- 4.5%. In all cases, Gal-3 expression in tumor cells was observed with variable staining pattern.Aside from GTR, no other clinical, histopathological, or immunohistochemical features were found to be related to the prognosis. We postulate that strict follow-up is recommended if piloA is associated with high mitotic activity/Ki67-LI, or if GTR cannot be achieved at surgery. Tumor recurrence or progression of the residual lesion should be strictly observed. In some aspects, childhood piloA remains an enigmatic tumor.

Cytogenetic studies of atypical choroid plexus papillomas (CPP) have been poorly described. In the present report, the cytogenetic investigation of an atypical CPP occurring in an infant is detailed. CPP chromosome preparations were analyzed by giemsa-trypsin-banding (GTG-banding) and comparative genome hybridization (CGH). Conventional karyotype analysis of tumor culture showed a normal chromosome complement. The results were confirmed by CGH, showing normal hybridization patterns for the sample. To date, the few atypical CPPs described in the literature have shown disparate cytogenetic information. This is the first report of a normal chromosome complement in atypical CPP. The heterogenic genetic features observed in these small series may reflect the diverse genetic background of choroid plexus tumors in children.

Meningiomas are recognized as the most common late complication following radiotherapy. However, cytogenetic studies in childhood atypical radiation-induced meningioma are sporadic, mainly because this condition generally occurs after a long latent period. In the present study we show the results of conventional and molecular cytogenetics in a 14-year-old boy with a secondary atypical meningioma. Apart from numerical changes, we found complex aberrations with the participation of chromosomes 1, 6 and 12. The invariable presence of loss of 1p was demonstrated by fluorescent in situ hybridization (FISH) analysis with probes directed to telomeric regions and by comparative genome hybridization (CGH). Previous cytogenetic studies on adult spontaneous and radiation-associated meningiomas showed loss of chromosome 22 as the most frequent change, followed by loss of the short arm of chromosome 1. To the best of our knowledge this is the first report of highly complex chromosome aberrations in the pediatric setting of meningioma.

Major skull defects, especially in the anterior region, can present as a most disturbing deformity. Reconstructive cranioplasty can restore cerebral protection and improve craniofacial aesthetics. Complex and large skull defects in children often present reconstructive difficulties.In this paper, an unusual case of a very large post-traumatic defect (318 cm(2)) in a child managed by a multidisciplinary team is described. The literature is reviewed.The management of a large complex skull defect in children is still controversial. Each case should be extensively debated in craniofacial units. In our case, bioceramics proved to be an effective and good alternative for final skull reconstruction.

KM+, a lectin purified from Artocarpus integrifolia seeds, is an attractant for neutrophils, and has properties similar to fMLP, IL-8 and MNCF. The endogenous lectin MNCF, inhibits carrageenan-induced neutrophil migration when intravenously administered in rats. In an attempt to mimic the activity of MNCF with KM+, we determined the effect of intravenous (iv) injection of KM+ (5 μg) on neutrophil migration to the peritoneal cavity of Wistar rats induced by KM+ (50 μg, intraperitoneal, ip), fMLP (5 ng, ip) and carrageenan (300 μg, ip). Initially we evaluated the effect of the time interval between intravenous and intraperitoneal administration of KM+. The intervals ranged from 20 to 120 min and progressively stronger inhibition was observed with increasing time intervals up to a maximum of 60 min, with effect decreasing thereafter. With injections at the optimum interval of 60 min, we observed that KM+ inhibited KM+- and carrageenan-induced neutrophil migration by 72%, and fMLP-induced migration by 56%. White cell counts for Wistar rats that only received KM+iv, performed at 0 to 120 min intervals after injection, revealed early neutropenia lasting 60 min, followed by a marked increase in circulating neutrophils that reached a maximum of twice the initial levels within 90 min and after 120 min returned to levels near to that observed before intravenous administration of KM+. These results indicate that when KM+ is present in the intravascular space, it produces an inhibitory effect on neutrophil migration similar to that caused by the intravenous administration of other chemoattractants, regardless of whether they act through a mechanism independent of carbohydrate recognition, as does IL-8, or are dependent on carbohydrate recognition, like MNCF.

The authors' aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere.Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3-15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case.The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.

Background Cerebrospinal fluid (CSF) abdominal pseudocyst is an uncommon but important complication of ventriculoperitoneal shunts. From the collected series, several features about the etiology and management become apparent.Retrospective datawere obtainedfrom12childrentreated with cerebrospinal fluid abdominal pseudocyst defined an alternative approach for management of these patients. Methods There were eight girls and four boys who ranged in age from 12 days to 18 years old (mean 7.7 months). The most frequent etiology of the hydrocephalus was Chiari type II malformation in six cases. Initial presentation with shunt malfunction was detected in nine cases. Abdominal distention and/or pain were the most frequent finding in our series (10/12). In only one case, infection was detected for all cerebrospinal fluid abdominal pseudocysts (8.3%). Ventriculoperitoneal shunt (VPS) externalization, antibiotics, and cyst aspiration was performed in 8/12 cases as initial management of abdominal pseudocyst. Ventriculoperitoneal shunt reinsertion in abdominal cavity and/or endoscopic third ventriculostomy (ETV) was performed as final management in 9/12. Ventriculoatrial shunt was used only in three cases. Recurrence of the abdominal cyst was observed in two cases. Discussion Based on the success rate (75%) in our series submitted to this management, a ventriculoperitoneal shunt can be safely reinserted in the majority of the patients. Endoscopic third ventriculostomy could be performed in selected cases as an alternative approach. Although infection has been reported as responsible for pseudocyst formation, it was only exceptionally found in our series.

Resective surgeries around eloquent areas challenge neurosurgeons and neuroimaging professionals due to difficulties to find anatomic references during surgery. Advances in magnetic resonance (MR) may prevent such deficits and provide information for accurate surgical planning. However, most of these techniques are expensive and not feasible in most centers. Using brain surface reformatted imaging (BSRI) of 3-D MR images, we sought to obtain data for surgical planning in patients with lesions around the motor cortex. Thirteen patients with lesions around the eloquent areas and considered for resective surgery were evaluated. Patients had different ages with tumors and malformative lesions. They were scanned in a 1.5-T Siemens magnet with volumetric sequence after injection of Gadolinium. Postprocessing was done in an auxiliary console using MRI station software. We performed reformatting as described by Hattingen et al. [J Neurosurg 102:302–310, 2005] and used fixed skull and vascular structures as anatomical references. Distances to the reference points were measured to allow surgical planning and locate sites for cortical stimulation. Patients were also studied by blood-oxygen-level-dependent functional magnetic resonance imaging to locate the hand area before the surgical procedure. All patients had cortical stimulation during the resective procedure or chronic electrode stimulation before surgery. There was concordance between data from functional and structural data. In one case, partial resection was performed due to the high risk of severe deficits. Even in this case, there were clinical improvements and no additional deficits were observed. BSRI may be a useful tool for surgical planning around eloquent areas. It can reduce surgical time and morbidity and showed correlation with functional studies. Added to this is the low cost and feasibility in most centers that have standard MR scanners with Multiplanar Reformatting software.

epd.2013.0571 Auteur(s) : Elvis Terci Valera1 elvisvalera@hotmail.com, Maria Sol Brassesco1, Helio Rubens Machado2, Ricardo Santos Oliveira2, Vera Cristina Terra3, Carlos Alberto Scrideli1, Luiz Gonzaga Tone1 1 Hospital das Clinicas da Faculdade de Medicina de Ribeirao Preto-USP 2 Division of Neurosurgery 3 Department of Neurology, Division of Epilepsy, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil To the Editor, Type II focal cortical dysplasia (FCD) corresponds to a specific malformation [...]

We report three patients with a history of maternal valproate use during pregnancy who presented with a combination of metopic suture synostosis and upper limb malformations, which could be diagnosed as Baller-Gerold syndrome (BGS). The patients underwent surgical treatment for the craniofacial deformity, during which standard frontocranial reconstruction was performed.Only 32 patients have been reported in the world literature and these cases support the emerging view that BGS is not a distinct syndrome, but should instead be considered to be an heterogeneous phenotype with variable etiology.Our case series suggests for the first time that fetal sodium valproate exposure may also cause this phenotype.

Object Hydrocephalus is a common disease process. Transcranial color-coded Doppler (TCCD) ultrasonography is an accepted noninvasive method with which to quantify intracranial blood flow in adults and children. The authors studied the applications of TCCD ultrasonography and the alterations of the flow velocity of the cerebral arteries in children with hydrocephalus. Methods One hundred thirty-five children were divided into three groups: Group 1 comprised 40 infants with asymptomatic hydrocephalus who had well-functioning ventriculoperitoneal (VP) shunts; Group 2 comprised 10 children with symptomatic hydrocephalus who had malfunctioning shunts that were replaced; and Group 3 was a control group of 85 healthy infants. All patients underwent sequential measurements of cerebral blood flow (CBF) velocities (systolic and diastolic velocities) and resistivity index (RI). One group of patients underwent functional tests (compression of the anterior fontanelle and CO2 vasoreactivity) to determine hemodynamic changes in cerebral circulation. A significant statistical change in RI measurements, end diastolic CBF velocity, and percentage of change in RI was shown in patients with malfunctioning shunts, and in infants with a well-functioning VP shunt vasomotor reactivity was severely reduced. Conclusions Transcranial color-coded Doppler ultrasonography can be used to perform follow-up assessments of normal and malfunctioning shunts in children with hydrocephalus; the functional tests are a noninvasive tool for evaluating the cerebral compliance and the cerebral autoregulation in infants with hydrocephalus. The autoregulatory capacity may partly or completely be lost in cases of long-term shunt-treated hydrocephalus, and loss of cerebral vasoreactivity may be responsible for long-term deficits commonly observed in children, which help explain some of symptoms related to slit ventricles.

Although CT scans are being increasingly used for diagnosis and management for choanal atresia, we present a case in which this exam led to a misdiagnosis of encephalocele. This occurred because the nasal roof can be unossified until one year of age, a physiological variation, known as chondrocranium. As nasal malformations can be associated, this child underwent surgical procedure, and the absence of encephalocele was identified only intra-operatively. The purpose of this case-report is to stress the presence of chondrocranium as a variation of anatomy, and that diagnosis of choanal atresia should be performed with radiological and endoscopic exams associated.