Neurocirugía transicional: ¿una nueva subespecialidad? / Transitional neurosurgery: a new subspecialty?

Introducción: El proceso de transición de un paciente de la etapa pediátrica a la adulta es un proceso dinámico, complejo y planificado, que incluye la transferencia propiamente dicha de una institución pediátrica a una adulta. El aumento de la sobrevida de niños y adolescentes con patologías crónicas, la falta de acuerdos formales entre instituciones de salud y la falta de enfoque multidisciplinario de estos casos son los principales problemas a tener en cuenta. El objetivo del presente trabajo es describir y proponer una respuesta a las situaciones y dificultades que se encuentran en la actualidad en la salud pública durante el proceso de transición de pacientes pediátricos con patología neuroquirúrgica crónica y de pacientes adultos con patología congénita. Para tal fin se deben considerar factores sociales, económicos y comunicacionales. Material y Método: Estudio observacional, transversal y retrospectivo de pacientes con patología neuroquirúrgica transicional desde el 01 de enero de 2017 al 31 de diciembre de 2018. En total se revisaron las historias clínicas de 47 pacientes del Hospital "El Cruce". Resultados: De los 47 pacientes observados, con un rango etario entre 17 y 42 años, realizamos una división de éstos en 2 grupos: GRUPO 1: pacientes adultos operados en la edad pediátrica que requieren un seguimiento crónico de su patología de origen; y GRUPO 2: pacientes adultos con patología congénita. En el GRUPO 1, observamos 38 pacientes (24 masculinos y 14 femeninos) de los cuales 24 fueron operados y 14 no operados (solo seguimiento clínico). Dentro de este grupo, el 63% de los pacientes (n=29) presentó como su patología de base para su seguimiento la hidrocefalia. En el GRUPO 2, observamos a 9 pacientes (2 masculinos y 7 femeninos) de los cuales 7 fueron operados y 2 no operados. Todos los pacientes de este último grupo presentaron como diagnóstico de base un disrafismo espinal. Conclusión: Se debe lograr una transición planificada para el bienestar de los jóvenes con necesidades especiales de atención de salud. Actualmente no hay acuerdos interinstitucionales formales para el seguimiento y atención de estos pacientes.Consideramos que existe un grupo de pacientes que se beneficiarían con la creación de una nueva subespecialidad neuroquirúrgica: la neurocirugía transicional. La misma debería desarrollarse en hospitales generales, de alta complejidad, donde coincidan en el servicio de neurocirugía de adultos, neurocirujanos con formación pediátrica

Introduction: The transition process of a patient from pediatric to adult stage is a dynamic, complex, and planned process which, strictly speaking, includes the transfer from a pediatric to an adult institution. The increased rate of survival of children and adolescents with chronic pathologies, lack of formal agreements between health institutions and lack of multidisciplinary approach to these cases are the main issues to consider. The purpose of this paper is to describe and provide a response to situations and difficulties that currently exist in the public health during the transition process of pediatric patients with chronic neurosurgical pathology and adult patients with congenital pathology. For this purpose, communication, social, and economic factors must be considered. Methods: Observational, cross-sectional, and retrospective study of patients with transitional neurosurgical pathology from January 1, 2017, to December 31, 2018. In total, 47 patient's medical records were reviewed from "El Cruce" Hospital. Results: A total of 47 patients, with an age range between 17 and 42 years, were observed. We divided our study population into 2 groups: GROUP 1: adult patients who have been operated in pediatric age and require chronic follow-up of their origin pathology; and GROUP 2: adult patients with congenital pathology. In GROUP 1, we observed 38 patients (24 male and 14 female) of whom 24 were performed neurosurgery, and 14 were not. Within this group, 63% of the patients (n = 29) presented hydrocephalus as their basic pathology for monitoring. In GROUP 2, we observed 9 patients (2 male and 7 female) of whom 7 were operated and 2 were not. All patients in this last group presented spinal dysraphism as their basic diagnosis. Conclusion: A planned transition for the well-being of young patients with special health care needs must be achieved. Currently, there are no formal institutional agreements for the monitoring and care of these patients. We believe that there is a group of patients who would benefit from the creation of a new neurosurgical sub-specialty: transitional neurosurgery. It should be developed in high complexity general hospitals, where neurosurgeons with pediatric training could be part of a general neurosurgery department.

NetPyNE, a tool for data-driven multiscale modeling of brain circuits.

Biophysical modeling of neuronal networks helps to integrate and interpret rapidly growing and disparate experimental datasets at multiple scales. The NetPyNE tool (www.netpyne.org) provides both programmatic and graphical interfaces to develop data-driven multiscale network models in NEURON. NetPyNE clearly separates model parameters from implementation code. Users provide specifications at a high level via a standardized declarative language, for example connectivity rules, to create millions of cell-to-cell connections. NetPyNE then enables users to generate the NEURON network, run efficiently parallelized simulations, optimize and explore network parameters through automated batch runs, and use built-in functions for visualization and analysis - connectivity matrices, voltage traces, spike raster plots, local field potentials, and information theoretic measures. NetPyNE also facilitates model sharing by exporting and importing standardized formats (NeuroML and SONATA). NetPyNE is already being used to teach computational neuroscience students and by modelers to investigate brain regions and phenomena.

[Foreign accent syndrome after aneurysmal disappearance due to AVM treatment]. / Remisión de aneurisma luego de exéresis de MAV con aparición de síndrome del acento extranjero.

INTRODUCTION: Aneurysms associated with arteriovenous malformations (AVM) are vascular lesions are usually found in up to 15% of cases, increasing the overall risk of bleeding. The behavior against associated aneurysms is dichotomous while there are reports of disappearance of the same after the removal of the AVM; other items emphasize early treatment. Foreign accent syndrome is a rare neurological disorder in which the patient speaks his mother language as would a foreigner and sounds as foreign "accent" to native listeners. OBJECTIVE: To report a patient who developed foreign accent syndrome after excision of an AVM and the evolution of an associated aneurysm. CASE PRESENTATION: A pediatric patient after a resection of fronto-opercular AVM referred back completely on an aneurysm associated hyper flow, presented with postsurgical foreign accent syndrome. CONCLUSION: A rare syndrome and spontaneous resolution of a proximal aneurysm after excision of an AVM is hereby reported.

Bactericidal effect of γ-radiation with 137Cesium in platelet concentrates.

INTRODUCTION: γ-radiation is a method that was originally designed for inactivation of T lymphocytes in blood and blood components in order to prevent transfusion associated-graft versus host disease (TA-GVHD). Klebsiella pneumoniae, Escherichia coli, Enterococcus faecium and Staphylococcus epidermidis strains are important pathogens in blood banks since they have been related to post-transfusional sepsis. This study was conducted to demonstrate that γ-radiation is effective in reducing the viability of bacteria in platelet concentrates (PC). MATERIAL AND METHODS: Klebsiella pneumoniae, E. coli, E. faecium and S. epidermidis strains were adjusted at 101 to 106 CFU/ml and used in artificial contamination assays in PC. Contaminated platelet concentrates were subjected to γ-radiation with doses of 2500 cGy in a 137Cesium irradiator. The average of surviving bacteria at different bacterial concentrations, logarithmic reduction values (LRV) and bacterial death after γ-radiation percentage was calculated. RESULTS: Escherichia coli and K. pneumoniae were eliminated in 101 to 103 CFU; in contrast with 104 to 106 CFU, the LRV were 2.4, 2.6 and 2.6 for E. coli and 3.3, 2.7 and 3.0 for K. pneumoniae strains at 104, 105 and 106 CFU respectively. For Gram-positive strains, 101 CFU in PC, the inactivation post γ-radiation was not completed. Logarithmic reduction values post γ-radiation were 0.8 to 1.2 for E. faecium and S. epidermidis strains respectively. CONCLUSION: γ-radiation cannot be an alternative for the inactivation of pathogens in PC, because of the bacterial concentration and pathogen nature - being resistant to γ-radiation, the Gram-positive bacteria.

Remisión de aneurisma luego de exéresis de MAV con aparición de síndrome del acento extranjero / Aneurysm remission after MAV surgery with appearance of foreign accent syndrome

Introducción: Los aneurismas asociados a malformaciones arteriovenosas (MAV) son lesiones vasculares que suelen encontrarse hasta en el 15% de los casos, incrementando el riesgo global de hemorragia. La conducta frente a los aneurismas asociados es dicotómica en la literatura, mientras existen reportes de la desaparición de los mismos luego de la exéresis de la MAV, otros artículos enfatizan su tratamiento precoz. El síndrome del acento extranjero es un raro trastorno neurológico en el que el paciente habla su lengua materna como lo haría una persona extranjera y suena con “acento” extranjero a oídos de los oyentes nativos. Objetivo: Presentar un paciente que desarrolla el síndrome del acento extranjero posterior a la exéresis de una MAV y la evolución de un aneurisma asociado. Presentación de caso: Paciente pediátrico que luego de la exéresis de una MAV fronto-opercular posterior izquierda remite por completo un aneurisma de hiperflujo asociado, presentando en el postquirúrgico el síndrome del acento extranjero.Conclusión: Queda reportado el caso de este raro síndrome y la resolución espontánea de un aneurisma proximal luego de la exéresis de una MAV.

Introduction: The aneurysms associated with arteriovenous malformations (AVM) are vascular lesions that are usually found in up to 15% of cases, increasing the overall risk of bleeding. The behavior against associated aneurysms is dichotomous while there are reports of the disappearance of the same after the removal of the AVM, other items emphasize early treatment. Foreign accent syndrome is a rare neurological disorder in which the patient speaks his mother language as you would a foreigner and sounds with foreign "accent" to native listeners.Objective: To report a patient who developed foreign accent syndrome after excision of an AVM and the evolution of an associated aneurysm.Case presentation: pediatric patient that after a resection of fronto-opercular AVM refers back completely on an aneurysm associated hyper flow, presenting postsurgical foreign accent syndrome.Conclusion: the case of this rare syndrome and spontaneous resolution of a proximal aneurysm after excision of an AVM is hereby reported.

[Cerebrovascular disease in childhood. Case series]. / Enfermedad cerebrovascular en la infancia: presentación de casos clínicos.

Stroke in childhood is considered rare. It may be ischemic or hemorrhagic. Its presentation is often acute and it is a leading cause of mortality in pediatrics. The aim of our work is to present the general features of stroke of 18 patients under 15 years of age admitted to the Hospital El Cruce, between July 2009 and June 2014, and their clinical outcome a year later. We observed a predominance of male patients and the median age of 5 years. The main clinical features were hemiparesis, seizures, headache and vomiting and sensory impairment. The most frequent type was ischemic and the middle cerebral artery territory was the most commonly involved. Twelve patients had no sequelae. The signs and symptoms were guiding stroke, neuroimaging studies were essential for diagnosis and patients evolved favorably with low number of recurrence and death.

La enfermedad cerebrovascular en la infancia es poco frecuente. Su presentación es aguda y constituye una de las 10 causas de mortalidad en pediatría. El objetivo de nuestro estudio es presentar las características de la enfermedad de 18 pacientes menores de 15 años ingresados en el Hospital El Cruce entre julio de 2009 y junio de 2014, y su evolución clínica al año del evento. Observamos un predominio de sexo masculino y una mediana de edad de 5 años. La forma de presentación en orden de frecuencia fue hemiparesia, convulsiones, cefalea y vómitos, y deterioro del sensorio. La forma isquémica fue la más frecuente y el territorio más afectado fue el de la arteria cerebral media. Doce pacientes no presentaron secuelas. Los signos y síntomas fueron orientadores de enfermedad cerebrovascular; los estudios de neuroimágenes, esenciales para su diagnóstico; y los pacientes evolucionaron favorablemente con bajo número de recurrencia y fallecidos.

Enfermedad cerebrovascular en la infancia: presentación de casos clínicos / Cerebrovascular disease in childhood: Case series

La enfermedad cerebrovascular en la infancia es poco frecuente. Su presentación es aguda y constituye una de las 10 causas de mortalidad en pediatría. El objetivo de nuestro estudio es presentar las características de la enfermedad de 18 pacientes menores de 15 años ingresados en el Hospital El Cruce entre julio de 2009 y junio de 2014, y su evolución clínica al año del evento. Observamos un predominio de sexo masculino y una mediana de edad de 5 años. La forma de presentación en orden de frecuencia fue hemiparesia, convulsiones, cefalea y vómitos, y deterioro del sensorio. La forma isquémica fue la más frecuente y el territorio más afectado fue el de la arteria cerebral media. Doce pacientes no presentaron secuelas. Los signos y síntomas fueron orientadores de enfermedad cerebrovascular; los estudios de neuroimágenes, esenciales para su diagnóstico; y los pacientes evolucionaron favorablemente con bajo número de recurrencia y fallecidos.

Stroke in childhood is considered rare. It may be ischemic or hemorrhagic. Its presentation is often acute and it is a leading cause of mortality in pediatrics. The aim of our work is to present the general features of stroke of 18 patients under 15 years of age admitted to the Hospital El Cruce, between July 2009 and June 2014, and their clinical outcome a year later. We observed a predominance of male patients and the median age of 5 years. The main clinical features were hemiparesis, seizures, headache and vomiting and sensory impairment. The most frequent type was ischemic and the middle cerebral artery territory was the most commonly involved. Twelve patients had no sequelae. The signs and symptoms were guiding stroke, neuroimaging studies were essential for diagnosis and patients evolved favorably with low number of recurrence and death.

Monitoreo de la presión intracraneana en paciente pediátricos con traumatismo de cráneo grave / Monitoring of intracranial pressure in pediatric patients with severe head trauma

El aumento y necesidad de control de la presión intracraneana (PIC) está asociado a diversas entidades clínico-quirúrgicas (edema cerebral, hemorragias, control postoperatorio de tumores, etc.) entre las cuales el traumatismo encefalocraneano (TEC) ocupa el lugar más importante. El TEC representa un grave problema sanitario a nivel mundial ya que constituye una de las causas más frecuentes de muerte en la edad pediátrica, siendo además la principal causa de retraso mental, epilepsia e incapacidad física. Hemos podido observar múltiples causas del TEC: obstétricas, caídas del niño, caída de la madre, accidentes pedestres y vehiculares, accidentes deportivos (fútbol, rugby, etc.), misceláneas (elementos arrojados, juguetes voladores, etc.), mordeduras de animales.

Munc18-1 controls SNARE protein complex assembly during human sperm acrosomal exocytosis.

The spermatozoon is a very specialized cell capable of carrying out a limited set of functions with high efficiency. Sperm are then excellent model cells to dissect fundamental processes such as regulated exocytosis. The secretion of the single dense-core granule of mammalian spermatozoa relies on the same highly conserved molecules and goes through the same stages as exocytosis in other types of cells. In this study, we describe the presence of Munc18-1 in human sperm and show that this protein has an essential role in acrosomal exocytosis. We observed that inactivation of endogenous Munc18-1 with a specific antibody precluded the stabilization of trans-SNARE complexes and inhibited acrosomal exocytosis. Addition of recombinant Munc18-1 blocked secretion by sequestering monomeric syntaxin, an effect that was rescued by α-soluble NSF attachment protein. By electron microscopy, we observed that both the anti-Munc18-1 antibody and recombinant Munc18-1 inhibited the docking of the acrosome to the plasma membrane. In conclusion, our results indicate that Munc18-1 plays a key role in the dynamics of trans-SNARE complex assembly and/or stabilization, a process that is necessary for the docking of the outer acrosomal membrane to the plasma membrane and subsequent fusion pore opening.

α-SNAP prevents docking of the acrosome during sperm exocytosis because it sequesters monomeric syntaxin.

α-SNAP has an essential role in membrane fusion that consists of bridging cis SNARE complexes to NSF. α-SNAP stimulates NSF, which releases itself, α-SNAP, and individual SNAREs that subsequently re-engage in the trans arrays indispensable for fusion. α-SNAP also binds monomeric syntaxin and NSF disengages the α-SNAP/syntaxin dimer. Here, we examine why recombinant α-SNAP blocks secretion in permeabilized human sperm despite the fact that the endogenous protein is essential for membrane fusion. The only mammalian organism with a genetically modified α-SNAP is the hyh mouse strain, which bears a M105I point mutation; males are subfertile due to defective sperm exocytosis. We report here that recombinant α-SNAP-M105I has greater affinity for the cytosolic portion of immunoprecipitated syntaxin than the wild type protein and in consequence NSF is less efficient in releasing the mutant. α-SNAP-M105I is a more potent sperm exocytosis blocker than the wild type and requires higher concentrations of NSF to rescue its effect. Unlike other fusion scenarios where SNAREs are subjected to an assembly/disassembly cycle, the fusion machinery in sperm is tuned so that SNAREs progress uni-directionally from a cis configuration in resting cells to monomeric and subsequently trans arrays in cells challenged with exocytosis inducers. By means of functional and indirect immunofluorescense assays, we show that recombinant α-SNAPs--wild type and M105I--inhibit exocytosis because they bind monomeric syntaxin and prevent this SNARE from assembling with its cognates in trans. Sequestration of free syntaxin impedes docking of the acrosome to the plasma membrane assessed by transmission electron microscopy. The N-terminal deletion mutant α-SNAP-(160-295), unable to bind syntaxin, affects neither docking nor secretion. The implications of this study are twofold: our findings explain the fertility defect of hyh mice and indicate that assembly of SNAREs in trans complexes is essential for docking.

Combination of CpG-oligodeoxynucleotides with recombinant ROP2 or GRA4 proteins induces protective immunity against Toxoplasma gondii infection.

Synthetic oligodeoxynucleotides containing unmethylated CpG motifs (CpG-ODN) have been characterized as Th1-promoting immunopotentiators, an adjuvant activity desirable for vaccination against intracellular parasites like Toxoplasma gondii. In an attempt to find new antigen-adjuvant combinations that enhance the immunogenicity of antigen candidates for toxoplasma vaccines, we analyzed the extent of protection in mice immunized with ROP2 and GRA4 recombinant proteins when co-administered with CpG-ODN. Both GRA4+CpG-ODN and ROP2+CpG-ODN formulations were shown to induce a strong humoral Th1-biased response characterized by a high IgG(2a) to IgG(1) antibody ratio. Both vaccination regimens led to increased secretion of IFN-γ and IL-10, and negligible amounts of IL-4, upon specific re-stimulation of spleen cells from these groups of mice. After a non-lethal challenge with tissue cysts of a moderately virulent strain, only the brains from mice vaccinated with ROP2 or GRA4 in combination with CpG-ODN showed a significant reduction (63% and 62%, respectively) in their parasite load compared to the controls. The rate of protection obtained with GRA4+ROP2+CpG-ODN resulted equivalent (66%) to those achieved with the single antigens plus CpG-ODN. Taken together, these results indicate that CpG-ODN is an important candidate adjuvant for use in potential multicomponent anti-T. gondii vaccines for animals and humans.

Sperm from hyh mice carrying a point mutation in alphaSNAP have a defect in acrosome reaction.

Hydrocephalus with hop gait (hyh) is a recessive inheritable disease that arose spontaneously in a mouse strain. A missense mutation in the Napa gene that results in the substitution of a methionine for isoleucine at position 105 (M105I) of alphaSNAP has been detected in these animals. alphaSNAP is a ubiquitous protein that plays a key role in membrane fusion and exocytosis. In this study, we found that male hyh mice with a mild phenotype produced morphologically normal and motile sperm, but had a strongly reduced fertility. When stimulated with progesterone or A23187 (a calcium ionophore), sperm from these animals had a defective acrosome reaction. It has been reported that the M105I mutation affects the expression but not the function of the protein. Consistent with an hypomorphic phenotype, the testes and epididymides of hyh mice had low amounts of the mutated protein. In contrast, sperm had alphaSNAP levels indistinguishable from those found in wild type cells, suggesting that the mutated protein is not fully functional for acrosomal exocytosis. Corroborating this possibility, addition of recombinant wild type alphaSNAP rescued exocytosis in streptolysin O-permeabilized sperm, while the mutant protein was ineffective. Moreover, addition of recombinant alphaSNAP. M105I inhibited acrosomal exocytosis in permeabilized human and wild type mouse sperm. We conclude that the M105I mutation affects the expression and also the function of alphaSNAP, and that a fully functional alphaSNAP is necessary for acrosomal exocytosis, a key event in fertilization.

Metástasis intracraneales en la infancia / Intracranial metastases in infant and childhood

Objective: To describe and analyze a series of pediatric patients who underwent surgery for intracranial metastasis at our department, specially considering the paucity of publications in the literature on the topic in this age group. Material and method: The study is a retrospective review of the clinical charts of patients with intracranial metastasis seen between 1988 and 2006. Inclusion and exclusion criteria were established for the different cases. Results: In this period, 1740 CNS tumors were operated on, of which 12 cases were intracranial metastasis (0.7). Mean age of the children was 11 years. Six patients were male and 6 female. Location of the primary tumor was: suprarenal in 4 case, in the bone in 2, and in the bladder, kidney, testicle, hypopharynx, facial bone, and thigh in 1 case each. Four patients had multiple metastasis: 10 located in the cerebral hemispheres, 2 in the skull bone, and 2 at the epidural level. All patients presented with symptoms due to the brain metastases. Total resection was achieved in 9 cases, subtotal resection in1, and partial resection in 2. Mortality rate was 50, with a mean follo-up of 23 months. Conclusion: The incidence rate of brain metastasis in children in much lower than the published incidence rates in adults. The location of the primary tumor and histology found were also different. We consider surgery, when possible, a good therapeutic option within the multimodal treatment of metastases.

Tratamiento neuroendoscopico de los quistes aracnoideos supratentoriales sintomáticos / Neuroendoscopy treatment of supratentorial arachnoid symptomatic cysts

Objective: Symptomatic supratentorial cysts are complicated lesion often associated with complex brain malformation and hydrocephalus. The traditional treatment was microsurgical technique or shunts. We present our experience of neuroendoscopic treatment of these lesions. Method: Between 1999 and 2007, 28 consecutive patients with symptomatic supratentorial cysts underwent neuroendoscopic treatment in our department. Results: The mean age was 3.1 years (range 1 month to 15 years), 18 were male and 10 female, In 5 patients a prenatal diagnosis was established. Eighteen patients presented increased intracranial pressure, 5 developmental delay, 3 seizures and 3 hemiparesis. According to its localization we have clasificated the cysts in: interhemispheric (8), intraventricular (7), cuadrigerminal (5), paraventricular (5) and suprasellar (3). We have excluded the temporal cysts of this series. All patients underwent neuroendoscopic treatment involving cystoventriculostomy in 24 and cystocisternostomy in 4. The complications were: 1 CFS leak, 3 patients required ventriculo-peritoneal shunt, 1 cystoperitoneal shunt and 1 subdural peritoneal shunt. There were no surgery-related morbidity or death. Conclusion: Endoscopic treatment of symptomatic supratentorial cysts can be considered a useful alternative to traditional treatment.

Presentación inusual de los quistes aracnoideos / Unusual presentation of arachnoid cysts

Objetivo: Describir y analizar la asociación entre quistes aracnoideos e higroma subdural. Esta es considerada una complicación excepcional, con sólo 24 casos reportados en la literatura, siendo de 5 casos la serie publicada más numerosa. Método. Se realizó el análisis retrospectivo de las historias clínicas de 5 pacientes portadores de quiste aracnoideo, en los que la forma de presentación consistió en higroma subdural e hipertensión endocraniana. Resultados. El rango de edad fue entre 3 y 15 años (promedio: 7 años). La relación M/F fue 3/2. En 3 casos los síntomas se presentaron después de un traumatismo encefalocraneal leve y en los 2 restantes fue espontáneo. Todos se manifestaron con síntomas de hipertensión encefalocraneana y edema de papila bilateral, en 2 de ellos se constató paresia del VI par izquierdo. A todos se le realizó TAC y en 2 casos IRM. En los 5 pacientes se constató un higroma subdural con efecto de masa y quiste aracnoideo de fosa media. Se realizó tratamiento quirúrgico de urgencia en los 5 casos. Todos los pacientes evolucionaron favorablemente con desaparición del higroma subdural. Actualmente se encuentran asintomáticos con un exament neurológico y fondo de ojo normal. Conclusión. Si bien el higroma subdural con hipertensión endocraneana en una complicación poco usual, debe ser tenida en cuenta como una de las formas de presentación clínica de los quistes aracnoideos, la cual requiere inmediata resolución quirúrgica. Palabras clave: complicaciones, higroma subdural, hipertensión endocraneana, quiste aracnoideo.(AU)

Objective: To analize and describe the association between arachnoid cysts and subdural hygroma. This association is an unusual complication of which only 24 cases have been reported in the literature. Methods: The clinical records of 5 patients with arachnoid cysts presenting as a subdural hygroma with increased intracranial pressure were analyzed retrospectively. Results: The ages of the patients ranged between 3 and 15 years-old (mean: 7 years-old). M/F ratio was 3/2. Onset of symptoms was postraumatic in 3 and spontaneous in 2. All patients presented symptoms of increased intracranial pressure and bilateral papilledema, and in 2 out of 5 paresis of the VI cranial nerve was found. All children underwent CAT scan and MRI was performed in 2. In all patients, subdural hygroma with mass effect and arachnoid cyst of the middle fossa was found. Emergency surgery was carried out in all cases. Al patients evolved favorably with complete disappearance of the subdural hygroma. They are currently asynptomatic with normal neurological examination and fundoscopy. Conclusion: Even though subdural hygroma with increased intracranial pressure is an unusual complication, it should be known as one of the clinical presentations of arachnoid cysts, which required immediate surgical intervention. Keywords: arachnoid syst, complications, increased intracranial pressure, subdural hygroma. (AU)

Presentación inusual de los quistes aracnoideos / Unusual presentation of arachnoid cysts

Objetivo: Describir y analizar la asociación entre quistes aracnoideos e higroma subdural. Esta es considerada una complicación excepcional, con sólo 24 casos reportados en la literatura, siendo de 5 casos la serie publicada más numerosa. Método. Se realizó el análisis retrospectivo de las historias clínicas de 5 pacientes portadores de quiste aracnoideo, en los que la forma de presentación consistió en higroma subdural e hipertensión endocraniana. Resultados. El rango de edad fue entre 3 y 15 años (promedio: 7 años). La relación M/F fue 3/2. En 3 casos los síntomas se presentaron después de un traumatismo encefalocraneal leve y en los 2 restantes fue espontáneo. Todos se manifestaron con síntomas de hipertensión encefalocraneana y edema de papila bilateral, en 2 de ellos se constató paresia del VI par izquierdo. A todos se le realizó TAC y en 2 casos IRM. En los 5 pacientes se constató un higroma subdural con efecto de masa y quiste aracnoideo de fosa media. Se realizó tratamiento quirúrgico de urgencia en los 5 casos. Todos los pacientes evolucionaron favorablemente con desaparición del higroma subdural. Actualmente se encuentran asintomáticos con un exament neurológico y fondo de ojo normal. Conclusión. Si bien el higroma subdural con hipertensión endocraneana en una complicación poco usual, debe ser tenida en cuenta como una de las formas de presentación clínica de los quistes aracnoideos, la cual requiere inmediata resolución quirúrgica. Palabras clave: complicaciones, higroma subdural, hipertensión endocraneana, quiste aracnoideo.(AU)

Objective: To analize and describe the association between arachnoid cysts and subdural hygroma. This association is an unusual complication of which only 24 cases have been reported in the literature. Methods: The clinical records of 5 patients with arachnoid cysts presenting as a subdural hygroma with increased intracranial pressure were analyzed retrospectively. Results: The ages of the patients ranged between 3 and 15 years-old (mean: 7 years-old). M/F ratio was 3/2. Onset of symptoms was postraumatic in 3 and spontaneous in 2. All patients presented symptoms of increased intracranial pressure and bilateral papilledema, and in 2 out of 5 paresis of the VI cranial nerve was found. All children underwent CAT scan and MRI was performed in 2. In all patients, subdural hygroma with mass effect and arachnoid cyst of the middle fossa was found. Emergency surgery was carried out in all cases. Al patients evolved favorably with complete disappearance of the subdural hygroma. They are currently asynptomatic with normal neurological examination and fundoscopy. Conclusion: Even though subdural hygroma with increased intracranial pressure is an unusual complication, it should be known as one of the clinical presentations of arachnoid cysts, which required immediate surgical intervention. Keywords: arachnoid syst, complications, increased intracranial pressure, subdural hygroma. (AU)

Presentación inusual de los quistes aracnoideos / Unusual presentation of arachnoid cysts

Objetivo: Describir y analizar la asociación entre quistes aracnoideos e higroma subdural. Esta es considerada una complicación excepcional, con sólo 24 casos reportados en la literatura, siendo de 5 casos la serie publicada más numerosa. Método. Se realizó el análisis retrospectivo de las historias clínicas de 5 pacientes portadores de quiste aracnoideo, en los que la forma de presentación consistió en higroma subdural e hipertensión endocraniana. Resultados. El rango de edad fue entre 3 y 15 años (promedio: 7 años). La relación M/F fue 3/2. En 3 casos los síntomas se presentaron después de un traumatismo encefalocraneal leve y en los 2 restantes fue espontáneo. Todos se manifestaron con síntomas de hipertensión encefalocraneana y edema de papila bilateral, en 2 de ellos se constató paresia del VI par izquierdo. A todos se le realizó TAC y en 2 casos IRM. En los 5 pacientes se constató un higroma subdural con efecto de masa y quiste aracnoideo de fosa media. Se realizó tratamiento quirúrgico de urgencia en los 5 casos. Todos los pacientes evolucionaron favorablemente con desaparición del higroma subdural. Actualmente se encuentran asintomáticos con un exament neurológico y fondo de ojo normal. Conclusión. Si bien el higroma subdural con hipertensión endocraneana en una complicación poco usual, debe ser tenida en cuenta como una de las formas de presentación clínica de los quistes aracnoideos, la cual requiere inmediata resolución quirúrgica. Palabras clave: complicaciones, higroma subdural, hipertensión endocraneana, quiste aracnoideo.

Objective: To analize and describe the association between arachnoid cysts and subdural hygroma. This association is an unusual complication of which only 24 cases have been reported in the literature. Methods: The clinical records of 5 patients with arachnoid cysts presenting as a subdural hygroma with increased intracranial pressure were analyzed retrospectively. Results: The ages of the patients ranged between 3 and 15 years-old (mean: 7 years-old). M/F ratio was 3/2. Onset of symptoms was postraumatic in 3 and spontaneous in 2. All patients presented symptoms of increased intracranial pressure and bilateral papilledema, and in 2 out of 5 paresis of the VI cranial nerve was found. All children underwent CAT scan and MRI was performed in 2. In all patients, subdural hygroma with mass effect and arachnoid cyst of the middle fossa was found. Emergency surgery was carried out in all cases. Al patients evolved favorably with complete disappearance of the subdural hygroma. They are currently asynptomatic with normal neurological examination and fundoscopy. Conclusion: Even though subdural hygroma with increased intracranial pressure is an unusual complication, it should be known as one of the clinical presentations of arachnoid cysts, which required immediate surgical intervention. Keywords: arachnoid syst, complications, increased intracranial pressure, subdural hygroma.

Pinealoblastomas in children.

INTRODUCTION: Series of pinealoblastomas (PB) usually comprise small number of cases as this tumor type is extremely rare and occurs mainly in childhood (especially under 9 years of age). Frequently, PB are reported together with others pineal parenchymal tumors (PPT) or pineal tumors, making characterization far from adequate. MATERIALS AND METHODS: Our series of CNS pediatric tumors comprises 1,350 cases of whom 16 are PPT, 12 PB, two pineocytomas (PC), and two mixed or transitional tumors (PC/PB). We have only analyzed the PB considering clinical features, treatment strategy, prognosis, recurrences, and mortality. RESULTS: PB represented 0.89%. Mean age was 7 years. Male-female ratio was 8/4. All patients complained of increased intracranial pressure, eight presented ocular symptoms, two cerebellar, and one endocrine disturbances. Patients underwent CT scans and/or MRI. All children had negative serum and CSF markers and only one case had positive tumor cells in the CSF on admission. Hydrocephalus (12/12) was treated with ventriculoperitoneal shunt in 11/12 and endoscopic third ventriculostomy (ETV) in 1/12. We performed 11 surgical procedures (seven by occipital transtentorial approach) and one endoscopic biopsy. Total removal was achieved in two, partial removal (50-90%) in seven, and biopsy in three patients or <50%. Adjuvant therapy included radiotherapy and chemotherapy. Recurrences appeared in 8/12 cases (mean time of recurrence=27.28 months). Six patients died (mean survival=29.55 months). Mean follow up for the six patients alive was 54 months and mean follow up for all 12 children was 38.7 months. CONCLUSION: In our opinion, PB have a poor prognosis and are very aggressive, especially in small children. Survival rate at 1 and 5 years in the present series is 66.6% (8/12) and 50% (6/12), respectively. We propose an algorithm for the treatment of pediatric patients with PB.