"The blue catheter" - case report.

The ventriculoperitoneal shunt placement procedure is common throughout the world, with a prevalence of approximately 30,000 procedures per year in the United States (5). However, the rate of complications is still important and must be remembered, with a percentage of 11 to 25% after the first year of implantation. Abdominal complications are described in 5-47% of the cases (6), and the abdominal pseudocyst is one of them. We present the case of a patient colonized by multidrug-resistant bacteria, who underwent exteriorization surgery of a ventriculoperitoneal shunt. Intraoperatively, a blue coloration was observed in the catheter, a phenomenon previously described in urinary infections (1)(2) but not before in pathologies of the central nervous system. Our objective is to present an atypical case of dysfunction of the ventriculoperitoneal shunt, whose diagnosis couldbe evidenced through the coloration of the catheter exteriorized in surgery.

TROPHY registry - status report.

INTRODUCTION: The TROPHY registry has been established to conduct an international multicenter prospective data collection on the surgical management of neonatal intraventricular hemorrhage (IVH)-related hydrocephalus to possibly contribute to future guidelines. The registry allows comparing the techniques established to treat hydrocephalus, such as external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). This first status report of the registry presents the results of the standard of care survey of participating centers assessed upon online registration. METHODS: On the standard of treatment forms, each center indicated the institutional protocol of interventions performed for neonatal post-hemorrhagic hydrocephalus (nPHH) for a time period of 2 years (Y1 and Y2) before starting the active participation in the registry. In addition, the amount of patients enrolled so far and allocated to a treatment approach are reported. RESULTS: According to the standard of treatment forms completed by 56 registered centers, fewer EVDs (Y1 55% Y2 46%) were used while more centers have implemented NEL (Y1 39%; Y2 52%) to treat nPHH. VAD (Y1 66%; Y2 66%) and VSGS (Y1 42%; Y2 41%) were used at a consistent rate during the 2 years. The majority of the centers used at least two different techniques to treat nPHH (43%), while 27% used only one technique, 21% used three, and 7% used even four different techniques. Patient data of 110 infants treated surgically between 9/2018 and 2/2021 (13% EVD, 15% VAD, 30% VSGS, and 43% NEL) were contributed by 29 centers. CONCLUSIONS: Our results emphasize the varying strategies used for the treatment of nPHH. The international TROPHY registry has entered into a phase of growing patient recruitment. Further evaluation will be performed and published according to the registry protocol.

Perinatal outcomes after open fetal surgery for myelomeningocele repair: a retrospective cohort study.

OBJECTIVE: Describe outcomes of open fetal surgery for myelomeningocele (MMC) repair in two Brazilian hospitals and the impact of surgical experience on outcome. DESIGN: Retrospective cohort study. SETTING: Sao Paulo, Brazil. POPULATION: 237 pregnant women carrying a fetus with an open spinal defect. METHODS: Surgical details, and maternal and fetal outcomes collected from all patients. MAIN OUTCOME MEASURES: Analysis of surgical and perinatal outcome parameters. RESULTS: Total surgical time was 119 ± 7.6 minutes. Preterm labour occurred in 24.2%, premature rupture of membranes in 26.7%, placental abruption in 0.8%, need for a blood transfusion at delivery in 2.1%, and dehiscence at the repair site in 2.5%. Reversal of hindbrain herniation at birth occurred in 71.4%. There were no maternal deaths or severe maternal morbidities. The failure rate with the patient anaesthetised was 0.42% and perinatal mortality was 2.1% (three intrauterine demises and two neonatal deaths). Comparing results from our study in the first 3 years with the last 3 years demonstrated improvement in the total surgical time (121.2 ± 6.4 versus 118.5 ± 8.2 minutes, P = 0.005) and an increase in reversal of hindbrain herniation at birth (64.0 versus 77.1%, P = 0.042). CONCLUSION: Our open fetal surgical approach for MMC was effective and results were comparable to past studies. Improvements in surgical performance and perinatal outcome increased as the surgical team became more familiar with the procedure. FUNDING: The study was funded solely by institutional funds. TWEETABLE ABSTRACT: Brazilian experience of in utero open surgery for myelomeningocele repair.

Amniotic fluid lactic acid and matrix metalloproteinase-8 levels at the time of fetal surgery for a spine defect: association with subsequent preterm prelabour rupture of membranes.

OBJECTIVE: In utero fetal surgery to correct incomplete closure of the spinal cord lessens the extent of permanent damage but is associated with preterm prelabour rupture of membranes (PPROM). We determined whether compounds in amniotic fluid collected at the time of surgery predicted subsequent development of PPROM. DESIGN: Prospective study. SETTING: Hospitals in Sao Paulo, Brazil. POPULATION: Twenty-four consecutive pregnant women at 24-26 weeks of gestation seen between February and October 2017 with a singleton pregnancy underwent in utero surgery to correct an open spinal defect in their fetus. METHODS: Amniotic fluid was tested for lactic acid, matrix metalloproteinase 2 (MMP-2), MMP-8, MMP-9 and interleukin-6 (IL-6) by enzyme-linked immunosorbent assay. Clinical data were collected after completion of all laboratory studies. MAIN OUTCOME MEASURE: Amniotic fluid concentration of compounds in women with or without PPROM. RESULTS: Preterm prelabour rupture of membranes occurred in seven (29.2%) women. There were no differences in maternal age, gravidity, parity, race, history of caesarean sections or fetal gender between women with or without PPROM. Length of surgery, days of wound healing and length of hospital stay were also indistinguishable. The median concentrations of MMP-8 (1.7 versus 0.6 ng/ml; P = 0.0041) and lactic acid (7.1 versus 5.9 mm; P = 0.0181) were higher in women with PPROM. The amniotic fluid MMP-8 level was also negatively correlated with gestational age at delivery (Spearman r = -0.4217, P = 0.0319). CONCLUSION: Differences in susceptibility to develop PPROM are present before fetal surgery. An increase in anaerobic glycolysis, evidenced by the intra-amniotic lactic acid level, may enhance MMP-8 production and weaken maternal and fetal membranes. TWEETABLE ABSTRACT: Matrix metalloproteinase-8 and lactic acid in amniotic fluid predict preterm prelabour rupture of membranes.

Mesenchymal stem cell-like properties of CD133(+) glioblastoma-initiating cells

Glioblastoma is composed of dividing tumor cells, stromal cells and tumor initiating CD133(+) cells. Recent reports have discussed the origin of the glioblastoma CD133(+) cells and their function in the tumor microenvironment. The present work sought to investigate the multipotent and mesenchymal properties of primary highly purified human CD133(+) glioblastoma-initiating cells. To accomplish this aim, we used the following approaches: i) generation of tumor subspheres of CD133(+) selected cells from primary cell cultures of glioblastoma; ii) analysis of the expression of pluripotency stem cell markers and mesenchymal stem cell (MSC) markers in the CD133(+) glioblastoma-initiating cells; iii) side-by-side ultrastructural characterization of the CD133(+) glioblastoma cells, MSC and CD133(+) hematopoietic stem cells isolated from human umbilical cord blood (UCB); iv) assessment of adipogenic differentiation of CD133(+) glioblastoma cells to test their MSC-like in vitro differentiation ability; and v) use of an orthotopic glioblastoma xenograft model in the absence of immune suppression. We found that the CD133(+) glioblastoma cells expressed both the pluripotency stem cell markers (Nanog, Mush-1 and SSEA-3) and MSC markers. In addition, the CD133(+) cells were able to differentiate into adipocyte-like cells. Transmission electron microscopy (TEM) demonstrated that the CD133(+) glioblastoma-initiating cells had ultrastructural features similar to those of undifferentiated MSCs. In addition, when administered in vivo to non-immunocompromised animals, the CD133(+) cells were also able to mimic the phenotype of the original patient's tumor. In summary, we showed that the CD133(+) glioblastoma cells express molecular signatures of MSCs, neural stem cells and pluripotent stem cells, thus possibly enabling differentiation into both neural and mesodermal cell types

Successful everolimus therapy for SEGA in pediatric patients with tuberous sclerosis complex.

PURPOSE: Tuberous sclerosis complex (TSC) is associated with hamartomatous growths including subependymal giant cell astrocytomas (SEGAs). Although, SEGAs are slow-growing glioneuronal tumors, they represent a significant cause of morbidity and mortality due to the risk of sudden death from acute hydrocephalus. Neurosurgical resection has been the mainstay of therapy, since radiotherapy and chemotherapy were proved inefficient in those tumors. Recent studies support the use of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis and suggest it might represent a disease-modifying treatment for other aspects of tuberous sclerosis. METHODS: We describe the clinical and radiological progression of three pediatric patients with definitive diagnosis of TSC and SEGA, which have been treated with everolimus. RESULTS: Up to 34 % sustained SEGA decrease was observed in the three cases. All three patients have experienced seizure control and two of them have showed cognitive and behavioral improvement. Everolimus has been well tolerated by all. No severe adverse events have been observed to date. CONCLUSION: Everolimus offers significant promise in treating SEGAs. Studies are required to explore optimal therapy duration and management upon discontinuing therapy.

Profile of latex sensitization and allergies in children and adolescents with myelomeningocele in São Paulo, Brazil.

BACKGROUND: Latex allergy is an important cause of occupational allergy. In many countries the prevalence of latex allergy is still high and the profile of latex sensitization is unknown. OBJECTIVES: To evaluate the frequency of sensitization and allergy to latex in children and adolescents with myelomeningocele and to identify associated risk factors. METHODS: The study included 55 children and adolescents with myelomeningocele followed at a specialized center. In addition to a standard questionnaire and skin prick tests (SPTs) to aeroallergens and total latex, the patients underwent determination of total and specific serum IgE to latex and recombinant allergens. RESULTS: We observed a prevalence of 25% for latex sensitization and of 20% for latex allergy. Twenty-four patients (43.6%) were atopic and the average age for the first reaction to latex was 44.5 months. Cutaneous reactions were the most frequently reported reactions (72.7%). Specific immunoglobulin (Ig) E to rHev b 1, rHev b 3, rHev b 5, rHev b 6.1, and rHev b 6.2 was detected in over 50% of patients allergic to latex. Multivariate analysis showed current asthma, atopy, and having undergone 4 or more operations to be risk factors for latex sensitization. CONCLUSIONS: Our study documented a high prevalence of sensitization and allergy to latex in patients with myelomeningocele. Specific IgE to rHev b 1, rHev b 3, rHev b 5, rHev b 6.1, and rHev b 6.2 was detected in over 50% of children and adolescents with myelomeningocele who are allergic to latex. A history of current asthma, atopy, and having undergone 4 or more operations were independent risk factors for latex allergy.

Profile of Latex Sensitization and Allergies in Children and Adolescents With Myelomeningocele in São Paulo, Brazil

Introducción: La alergia al látex constituye una causa importante de alergia ocupacional. En muchos países la prevalencia de alergia al látex es elevada y su perfil de sensibilización desconocido. Objetivos: El objetivo de este estudio fue evaluar la frecuencia de sensibilización y de alergia al látex en niños y adolescentes con mielomeningocele e identificar los factores de riesgo asociados. Métodos: Este estudio evaluó 55 niños y adolescentes afectos de mielomeningocele y monitorizados en un centro especializado. Se realizó un estudio de test cutáneos (prick) frente a aeroalérgenos y látex total. Así como un cuestionario y la determinación de IgE total y específica frente a látex y sus alérgenos recombinantes. Resultados: En los resultados encontramos un 25% de sensibilización y un 20% de alergia al látex. Un 43.6% de los pacientes eran atópicos y la edad media de comienzo de la sintomatología fue de 44.5 meses, siendo las reacciones cutáneas las más frecuentes (72.7%). La IgE específica frente a rHev b 1, 3, 5, 6.1 y 6.2 se detectó en más del 50% de los pacientes alérgicos al látex. El análisis multivariante mostró como significativas las siguientes variables: asma, atopia, y número de cirugías a las que había sido sometido el paciente. Conclusiones: Como conclusión este estudio documenta una elevada prevalencia de sensibilización y alergia al látex en pacientes con mielomeningocele. La IgE específica frente a rHev b 1, 3, 5, 6.1 y 6.2 se detecta en más del 50% de los alérgicos al látex en esta serie. Entre los factores independientes de riesgo para sufrir alergia a látex se encuentran el asma, la atopia y el haber estado sometido a más de cuatro cirugías (AU)

Background: Latex allergy is an important cause of occupational allergy. In many countries the prevalence of latex allergy is still high and the profile of latex sensitization is unknown. Objectives: To evaluate the frequency of sensitization and allergy to latex in children and adolescents with myelomeningocele and to identify associated risk factors. Methods: The study included 55 children and adolescents with myelomeningocele followed at a specialized center. In addition to a standard questionnaire and skin prick tests (SPTs) to aeroallergens and total latex, the patients underwent determination of total and specific serum IgE to latex and recombinant allergens. Results: We observed a prevalence of 25% for latex sensitization and of 20% for latex allergy. Twenty-four patients (43.6%) were atopic and the average age for the first reaction to latex was 44.5 months. Cutaneous reactions were the most frequently reported reactions (72.7%). Specific immunoglobulin (Ig) E to rHev b 1, rHev b 3, rHev b 5, rHev b 6.1, and rHev b 6.2 was detected in over 50% of patients allergic to latex. Multivariate analysis showed current asthma, atopy, and having undergone 4 or more operations to be risk factors for latex sensitization. Conclusions: Our study documented a high prevalence of sensitization and allergy to latex in patients with myelomeningocele. Specific IgE to rHev b 1, rHev b 3, rHev b 5, rHev b 6.1, and rHev b 6.2 was detected in over 50% of children and adolescents with myelomeningocele who are allergic to latex. A history of current asthma, atopy, and having undergone 4 or more operations were independent risk factors for latex allergy (AU)

A data mining system for providing analytical information on brain tumors to public health decision makers.

Cancer is the leading cause of death in economically developed countries and the second leading cause of death in developing countries. Malignant brain neoplasms are among the most devastating and incurable forms of cancer, and their treatment may be excessively complex and costly. Public health decision makers require significant amounts of analytical information to manage public treatment programs for these patients. Data mining, a technology that is used to produce analytically useful information, has been employed successfully with medical data. However, the large-scale adoption of this technique has been limited thus far because it is difficult to use, especially for non-expert users. One way to facilitate data mining by non-expert users is to automate the process. Our aim is to present an automated data mining system that allows public health decision makers to access analytical information regarding brain tumors. The emphasis in this study is the use of ontology in an automated data mining process. The non-experts who tried the system obtained useful information about the treatment of brain tumors. These results suggest that future work should be conducted in this area.

Frameless image-guided neuroendoscopy training in real simulators.

BACKGROUND: Over the last decade, neuroendoscopy has re-emerged as an interesting option in the management of intraventricular lesions in both children and adults. Nonetheless, as it has become more difficult to use cadaveric specimens in training, the development of alternative methods was vital. The aim of this study was to analyze the performance of a real simulator, in association with image-guided navigation, as a teaching tool for the training of intraventricular endoscopic procedures. METHODS: 3 real simulators were built using a special type of resin. 1 was designed to represent the abnormally enlarged ventricles, making it possible for a third ventriculostomy to be performed. The remaining 2 were designed to simulate a person's skull and brain bearing intraventricular lesions, which were placed as follows: in the foramen of Monro region, in the frontal and occipital horns of the lateral ventricles and within the third ventricle. In all models, MRI images were obtained for navigation guidance. Within the ventricles, the relevant anatomic structures and the lesions were identified through the endoscope and compared with the position given by the navigation device. The next step consisted of manipulating the lesions, using standard endoscopic techniques. RESULTS: We observed that the models were MRI compatible, easy and safe to handle. They nicely reproduced the intraventricular anatomy and brain consistence, as well as simulated intraventricular lesions. The image-based navigation was efficient in guiding the surgeon through the endoscopic procedure, allowing the selection of the best approach as well as defining the relevant surgical landmarks for each ventricular compartment. Nonetheless, as expected, navigation inaccuracies occurred. After the training sessions the surgeons felt they had gained valued experience by dealing with intraventricular lesions employing endoscopic techniques. CONCLUSION: The use of real simulators in association with image-guided navigation proved to be an effective tool in training for neuroendoscopy.

Tratamento da doença renal crônica: estratégias para o maior envolvimento do paciente em seu auto-cuidado / Chronic kidney disease treatment: strategies for increasing the patient’s participation in self-care

A identificação de pacientes portadores de doença renal crônica (DRC) ou em risco para o seu desenvolvimento pode ser feita por meio de testes simplesna rotina clínica. A importância do diagnóstico precoce da DRC está na possibilidade de instituição de intervenções clínicas que contribuem para o retardo da progressão da doença, postergando o início da terapia renal substitutiva e, portanto, trazem ganhos para a qualidade de vida do paciente, contribuindo com redução de custos do sistema público de saúde. No entanto, uma parcela importante dos pacientes é encaminhada tardiamente ao nefrologista. Mesmo com o encaminhamento tardio, o tratamento da doença permite o controle da condição clínica do paciente. As diversas complicações que ocorremao longo da evolução da DRC chamam atenção para a importância do tratamento especializado e multifacetado, realizado por equipe interdisciplinar. A baixa adesão dos pacientes ao tratamento de doenças crônicas reforça a importância desse tipo de abordagem. Neste sentido, o presente trabalho tem como objetivos apresentar estratégias que possam envolver mais os pacientes no seu auto-cuidado, aumentar a adesão ao tratamento e otimizar o trabalhoda equipe interdisciplinar.

Simple and cost-effective laboratorial parameters can be used by the clinical practitioner for screening and diagnosing chronic kidney disease (CKD). The importance of diagnosing CKD, still in early stages, lies in the possibility of delaying progression of the disease, postponing the beginning of renal replacement therapy, and therefore, improving the patient’s quality of life and lowering costs of the public health care system. In addition, early detection of CKD allows for better control of the disturbances commonly developed as renal function decreases. However, an important proportion of patients are referred to the nephrologists only when the kidney function is markedly decreased. Even considering this late referral, treatment before the beginning of renal replacement therapy allows for better control of the patient’s overall clinical state. Such treatment can achieve better results when a multidisciplinary team is present. This is particularly important when considering multiple aspects of CKD and the low adherence of the patients to the treatment of chronic diseases in general. Accordingly, this study proposes a series of strategies to enhance the patient’s adherence to the treatment and, ultimately, improve the results and patient outcome.

Apoptosis in alpha interferon (IFN-alpha) intratumoral chemotherapy for cystic craniopharyngiomas.

OBJECTIVES: The aim of this study was to verify whether intracystic injections of alpha-Interferon (IFN-alpha) in cystic craniopharyngiomas were able to reduce the tumor by activating the Fas apoptotic pathway. MATERIALS AND METHODS: Twenty-one patients with cystic craniopharyngiomas treated at the Pediatric Oncology Institute (IOP/GRAACC) of Federal University of São Paulo were submitted to intracystic chemotherapy with IFN-alpha. The tumor sizes of all patients were monitored and the apoptotic factor soluble FasL (sFasL) concentration was determined by ELISA (enzyme-linked immunosorbent assay) in tumor fluid samples from eight patients. RESULTS: There was a complete reduction in 11 patients, a partial response in seven, and a minor response in three patients. The concentration of sFasL was increased in all the eight patients examined concomitantly with the tumor size reduction. CONCLUSIONS: Our data demonstrated that the IFN-alpha was able to induce Fas-mediated apoptosis together with a reduction in the tumor size; such an observation may suggest the importance to investigate still unexplored mechanisms to be exploited in craniopharyngioma therapy.

Use of interferon alpha in intratumoral chemotherapy for cystic craniopharyngioma.

OBJECTIVES: This study analyzed the intratumoral activity of interferon alpha (IFN-alpha) in the treatment of cystic craniopharyngiomas. PATIENTS AND METHODS: From January 2000 to January 2004, nine patients presenting with cystic craniopharyngiomas were treated with intratumoral injection of IFN-alpha at the Pediatric Oncology Institute of the Federal University of São Paulo-Escola Paulista de Medicina. Age ranged from 1 year and 10 months to 18 years (mean 10 years). All intratumoral catheters were inserted by a subfrontal approach. Doses varied from 36 to 108 MU. RESULTS: There was complete disappearance of the lesion in seven cases. In two cases, partial reduction of tumor size was observed at follow-up. Follow-up varied from 1 year to 3 years and 6 months (mean 1 year 8 months). CONCLUSIONS: IFN-alpha proved to be an effective drug in the control of cystic craniopharyngiomas. Additional studies should be carried out to determine the optimal dose of IFN-alpha in the treatment of cystic craniopharyngioma. In addition, other drugs possessing high efficacy and low neurotoxicity should be analyzed.

Intracranial haemorrhage among a population of haemophilic patients in Brazil.

Intracranial haemorrhage (ICH) is a common cause of morbidity and mortality in haemophilic patients. The overall incidence of ICH has been reported to range from 2.2% to 7.5% in patients with haemophilia. From 1987 to 2001, 401 haemophilic patients from the Serviço de Hemofilia, Disciplina de Hematologia e Hemoterapia, Universidade Federal de São Paulo were evaluated. The episodes of ICH were documented by CT scan and the anatomic location, clinical presentation, relationship to trauma and clinical factors, including the presence of HIV infection and the presence of inhibitor, were reviewed. Among 401 haemophilic patients, 45 ICH episodes in 35 (8.7%) patients with age ranging from 4 days to 49 years (mean 10.6 years) were observed. A history of recent trauma was documented in 24 (53.3%) cases. Seventeen (37.8%) episodes occurred in more than one site of bleeding, 12 (26.7%) were subdural, seven (15.5%) subarachnoid, four (8.9%) epidural, two (4.4%) intracerebral and one (2.2%) intraventricular. The most frequent symptoms were headache and drowsiness. All patients were submitted to replacement therapy and neurosurgical intervention was performed in eight (17.8%) patients. Despite the treatment, three (8.6%) haemophilia A patients died due to the ICH event and three presented late sequelae. The most important aspect of ICH management is the early replacement therapy in haemophilic patients. This prompt treatment will increase the chances of a better prognosis. Another impact measure consists in the administration of the deficient coagulation factor after every head trauma, even when considered minor.

Endovascular treatment of intracavernous carotid aneurysm with hyperprolactinaemia.

SUMMARY: A 42-year-old woman presented with headache, galactorrhoea, marked hyperprolactinemia and normal neurologic examination. CT, MR and MR-angiography showed an intracavernous carotid artery aneurysm with minimal displacement of the pituitary stalk. Treatment by embolisation using GDC coils allowed exclusion of the aneurysm. Galactorrhoea resolved and serum prolactin levels fell to normal after eight months. Followup MR showed absence of the aneurysm, although the discrete mass effect persisted, suggesting that hyperprolactinaemia was due to pulsatility.

Use of bleomycin in intratumoral chemotherapy for cystic craniopharyngioma. Case report.

The authors present a case of a gigantic cystic craniopharyngioma that was treated with intratumoral injections of bleomycin. The mass had eroded the skull base and extended to the sphenoid bone. A total of eight intratumoral injections through an Ommaya reservoir were given. Six months after treatment, there was complete regression of the lesion and improvement in both visual and endocrinological symptomatology.

Cranioectodermal dysplasia with sagittal craniosynostosis (Sensenbrenner's syndrome): case report and review of the literature.

We report the clinical features and neurosurgical management of a 9-month-old girl with cranioectodermal dysplasia and scaphocephaly. The technique of surgical correction is discussed together with details of the pre-operative investigations. The importance of early referral to an experienced geneticist is emphasized.

Skull base in trigonocephaly.

The authors present aspects of the skull base in trigonocephalic children. Different patterns on clinical anthropometric investigation, bidimensional computer tomography (CT) scan and three-dimensional (3D) CT scan were studied. We present a series of 27 cases of trigonocephaly operated on in the department of pediatric neurosurgery at the Hôpital des enfants La Timone in Marseille since 1975. The skull base has been studied with CT scan in 12 patients and in 5 a 3D CT reconstruction was performed. A control series of 27 children without synostosis has been studied with the same patterns. We have analyzed in 3D, the volume of the anterior fossa related to the orbital volume, the permeability of basal sutures and the spatial orientation of petrous, sphenoid and zygomatic bones. An analysis of nasoethmoidal complex was performed in relation with hypotelorism. CT scan allows the study of the opening of orbital, nasion-pterional angles and the clinopterional angles, as well as nasion-clinoidal and bipterional distances. A computerized analysis of these data compared with normal skull base permits a new approach to these malformations and the necessity for subdivision of trigonocephaly.