Algunos desafíos en mucopolisacaridosis tipo I / A few challenges in mucopolysaccharidosis type I

Se describen como desafíos actuales en mucopolisacaridosis I la necesidad de una clasificación adecuada, vinculándola a las indicaciones terapéuticas; el diagnóstico temprano desde la pesquisa neonatal, sus ventajas y dificultades hasta la sospecha clínica de las formas grave y atenuada; el cuidado de la patología espinal y oftalmológica, desde el diagnóstico, el seguimiento y el tratamiento; las reacciones alérgicas por terapia de reemplazo enzimático, su diagnóstico y tratamiento. Por último, la transición hacia el cuidado adulto

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care

Nuevas recomendaciones para el cuidado de los pacientes con mucopolisacaridosis tipo I / New recommendations for the care of patients with mucopolysaccharidosis type I

Dados los avances sobre mucopolisacaridosis Icon posterioridad al consenso publicado en la Argentina por un grupo de expertos en 2008, se revisan recomendaciones respecto a estudios genéticos, seguimiento cardiológico, cuidado de la vía aérea, alertas sobre aspectos auditivos, de la patología espinal y neurológica. Se hace revisión de la terapéutica actual y se enfatiza en la necesidad de un diagnóstico y tratamiento precoces, así como de un seguimiento interdisciplinario

Considering the advances made on mucopolysaccharidosis type I after the consensus study published by a group of experts in Argentina in 2008, recommendations about genetic testing, cardiological follow-up, airway care, hearing impairment detection, spinal and neurological conditions, as well as current treatments, were reviewed. Emphasis was placed on the need for early diagnosis and treatment, as well as an interdisciplinary follow-up

Aspectos neuroquirúrgicos de las mucopolisacaridosis / Neurosurgical aspects of mucopolysaccharidoses

Mucopolisacaridosis es una rara enfermedad que afecta al metabolismo de los mucopolisacaridos debida a la ausencia o deficiencia de las enzimas encargadas de su síntesis lo que produce depósitos de aminoglucósidos en casi todos los tejidos del organismo. De acuerdo a la enzima faltante se clasifican los distintos tipos de la enfermedad, siendo más frecuente el Tipo I con sus tres variantes: Hurler, Hurler-Sheie y Sheie de distinta gravedad y tratamiento. Al nacimiento el niño no presenta síntomas, éstos van apareciendo a partir del año de vida: retardo físico y mental, múltiples deformidades esqueléticas, hepatoesplenomegalia, sordera, opacidades corneanas, entre otras. La intervención neuroquirúrgica en esta entidad es en dos patologías: la hidrocefalia, que al no presentar los signos clásicos de hipertensión endocraneana puede ser confundida con atrofia y la compresión medular cervical por los depósitos de mucopolisacáridos en vértebras, ligamentos y leptomeninges.

Mucopolysaccharidosis is a rare illness that involves the metabolism of mucopolysaccharides, that due to the absence or deficiency of corresponding enzymes, accumulate in almost all the tissues of the body. According to which enzyme is missing, different types of the disease have been identified; the most frequent being Type I with its three variants: Hurler, Hurler-Sheie, and Sheie. Symptoms of this disorder progress and range from mental and physical retardation, multiple skeletal deformities, hepatosplenomegaly, deafness, and corneal opacities among others. Children affected usually appear normal at birth and the slowness in their development may be the first evidence of the disorder whose progression is downhill. Neurosurgical intervention occurs in two pathologies: hydrocephalus that does not show signs of intracranial hypertension and can be confused with atrophy, and cervical cord compression due to storage of mucopolysaccharides in vertebrae, ligaments, and leptomeninges.

Metástasis supratentorial de meduloblastoma de fosa posterior / Supratentorial metastasis of P-fosa medulloblastoma

Objective: our objective is to analyze the patients that presented supratentorial metastases. Material and methods: we studied 170 patients with medulloblastoma from the year 1991 to 2005. Twenty seven presentedsupratentorial metastases. We considered age at presentation, pathology, staging, dissemination to other places and outcome. Results: twenty seven patients (15,8%) with medulloblastoma presented supratentorial metastases, the average age was 5,87 years range from 3 to 11 years. 13/27 were staged high risk and 14/27 standard. The average time between surgery and presence of recurrence was 12,65 month. 12/27 presented desmoplasia. 25/27 patients died. Conclusion: 15,8% of patients with medulloblastoma presented supratentorial metastases before 16 months of the first surgery. Allthe patients were younger than 12 years. The appearance of supratentorial metastases is not related with the stage or the presence of desmoplasia. The outcome was unfavorable once the dissemination was diagnosed.

Fistulas piales cerebrales en pediatría / Pial shunts in pediatric patients

Objective: to analyze our experience in brain pial arteriovenous fistulae in paediatric patients and review the modern concepts about this pathology.Materials and method: between 2004 and 2011, 6 cases of pial arteriovenous fistulae were diagnosed and treated at the National Hospital of paediatrics “Juan P.Garrahan”, defining them as arteriovenous malformations with one or more arterial connections with one single venous drainage, without interposed nidus. We reviewed the medical records and neuroimaging. The main method for diagnosis was cerebral angiography. Results: there were 4 male and 2 female patients, ages ranging from 1 month to 14 years old. 3patients presented brain hemorrhage, 2 had seizures as the initial symptom, and one was diagnosed because of his congenital hydrocephalus. 2 were treated with open surgery and 4 with embolisation. There were no important complications or rebleeding. Conclusion: brain pial fistulas are infrequent vascular malformations important for their high risk of bleeding and relative frequency in paediatrics. The management of these patients needs a multidisciplinary team for analysis, discussion, choosing the most suitable treatment, and follow up.

Aneurismas cerebrales en la infancia: un solo nombre para diferentes enfermedades / Cerebral aneurysms in infancy: a name for different diseases

Objetivo. Resaltar la heterogeneidad de los aneurismas cerebrales en la población pediátrica. Material y método. 19 pacientes con 20 aneurismas intracraneanos fueron tratados en nuestro Hospital en los ultimos 6 años.Resultados. La edad media fue de 12 años (rango 10 meses a 17 años). 65 %de los aneurismas fueron saculares, 25% fusiformes, hubo un aneurisma infeccioso y otro lenticuloestriado distal. Los pacientes con aneurismas saculares fueron predominantemente varones y se manifestaron más comúnmente con hemorragia intracraneana (92%). Los aneurismas fusiformes se originaron posteriormente a una disección o fueron crónicos con trombo mural y ejerciendo efecto de masa. La terapéutica fue diferente según el tipo aneurismático. Conclusión. Los aneurismas pediátricos son un grupo heterogéneo de enfermedades arteriales intracraneanas con manifestaciones clínicas, morfología y terapéutica diferentes.

Aneurismas experimentales en conejos por angioplastia con balón / Experimental aneurysms in rabbits by ballon angioplasty

Objetive. To perform a new model of experimental aneurysms in rabbits and to achieve a training in basic endovascular techniques. Material and method. We introduce a new aneurysm model in rabbits. First we performed a balloon angioplasty in the right carotid artery in the neck, then three weeks later we carry out an angiography with a diagnostic catheter from the right femoral artery to check aneurysm patency. Results. We were able to perform 10 aneurysms in ten rabbits; they were patent three weeks after their creation. The aneurysmscreation and the angiography performed to evaluate the aneurysm patency required a similar skill necessary to perform basic endovascular interventions. Conclusion. Aneurysms created from balloon angioplasty in the carotid artery in rabbits are a suitable model at least in the short term. The process of aneurysm formation and its study is an useful training in basic endovascular techniques.

Patología vascular en pacientes menores de un año / Cerebrovascular pathology in infants

Objetivo. Analizar nuestra experiencia en patología neuroquirúrgica vascular en los pacientes menores de un año.Material y método. Entre el 1/1/89 y el 1/6/09 se trataron en el Servicio de Neurocirugía del Hospital Nacional de Pediatría Juan P. Garrahan 235 pacientes con malformación vascular del SNC. De ellos 19 (8%) eran menores de un año;11 fueron varones y 8 mujeres, con una edad media de 4,5 meses al diagnóstico, en un rango desde prenatal a 1 año. Se analizaron las historias clínicas y los estudios neurradiológicos de cada uno de ellos.Resultados. Todos los pacientes fueron estudiados con TAC inicial excepto las malformaciones aneurismáticas de la vena de Galeno (MAVG) que se diagnosticaron en un principio, con ecografía. En 16/19 pacientes se realizó angiografía digital cerebral, y en uno de los casos se efectuó angiografía espinal, hallándose la malformación a ese nivel. Los tipos de lesiones halladas fueron: 6 MAVG (4 coroideas y 2 murales), 6 malformaciones arteriovenosas (MAV), 2 fístulas arteriovenosas (A-V) piales (1 cerebral y 1 medular), 1 fístula A-Vmedular, 1 cavernoma, 1 aneurisma sacular y 2 malformaciones embrionarias en fosa posterior. Conclusión. Es remarcable la variabilidad y complejidad de las malformaciones vasculares del SNC en el primer año de vida. Es necesaria la integración del equipo endovascular al neuroquirúrgico, no sólo para el diagnóstico, sino también para completar o facilitar el tratamiento de alguna de estas lesiones. Las malformaciónes vasculares más frecuentes en este grupo son la MAVG y las MAV , seguidas de las fístulas A-V.

Objective. To analyze our experience in neurosurgical vascular disease in patients under one year. Materials and Methods. Between 1/1/89 and 1/6/09 we treated at the Department of Neurosurgery of the Hospital Nacional de Pediatría Juan P. Garrahan 235 patients with vascular malformation of the central nervous system. Of these19 (n = 19; 8%) were infants under one year, 11 were males and 8 females, mean age at diagnosis 4.5 months (range from prenatal to 1 year). We evaluated all charts and neuroradiologic studies of each patient. Results. All patients were studied with initial CT scan except one patient with Vein of Galen Aneurysmal Malformation (VGAM)who was diagnosed antenatal by ultrasound. The types of lesions were 6 VGAM (4 choroidal type and 2 mural type), 6 Arteriovenous Malformations (AVM), 2 Pial Arteriovenous Fistulas(1 brain and 1 spinal), 1 spinal Dural Arteriovenous Fistula, 1 Cavernoma, 1 Aneurysm and 2 Embryologic Dural Sinus Malformations at the posterior fossa. Conclusion. It is remarkable variability and complexity ofvascular malformations of the central nervous system in the first year of life. It is necessary to integrate the endovascular neurosurgical team, not only for diagnosis but also to complement or facilitate the treatment of any of theses vascular disease. The most frequent vascular malformations in our group were the VGAM and the AVM followed by AV Fistulas.

Uso de pericardio humano en neurocirugía / Use of human pericardium in neurosurgical practice

Objetivo. Describir y analizar una serie de pacientes operados en nuestro servicio por diferentes patologías, en los cuales se utilizó pericardio de donante no vivo para la realización de la duroplastía, obtenido del Banco de Tejidos de nuestro Hospital. Material y método. Se realizó un análisis retrospectivo de las historias clínicas de 33 pacientes en los cuales se utilizó pericardio humano, operados entre los años 2006 y 2008. Resultados. La edad de los pacientes fue entre 1 mes y 19 años (promedio: 6,6 años). 17 masculinos, 16 femeninos. Las cirugías en las cuales se utilizó, por diferentes motivos, pericardio humano para el cierre de la duramadre fueron: 7 cirugías por tumores de fosa posterior, 6 por tumores supratentoriales, 5 por traumatismos encefalocraneanos, 3 por malformación de Arnold-Chiari, 3 por hematomas espontáneos, 2 por malformaciones arteriovenosas, 2 por lipomas lumbosacros, 2 por encefaloceles, 1 por cirugía de la epilepsia, 1 por tumor espinal, y 1 por tumor de tronco. Las complicaciones encontradas fueron: 2 pseudomeningoceles y 1 fístula de líquido cefalorraquídeo, las que se resolvieron sin necesidad de nueva intervención quirúrgica. No se presentaron infecciones de la herida quirúrgica ni rechazo del injerto. Follow up: 18,6 meses. Conclusión. Consideramos que este material se comporta en forma excelente como sustituto de la duramadre, presentando ventajas tales como: ser impermeable, fácil de suturar y manejar, proporcionando un sellado hermético, baja antigenicidad y no inducir reacciones a cuerpo extraño.

Objective. To describe and to analyze a series of patients operated in our service by different pathologies, in which we used pericardium of alive donor for dural closure, stored at 80°Cbelow cero in our hospital. Method. A retrospective analysis was designed to evaluate the medical records of 33 patients in which human pericardiumwere used, in a period of time between 2006 and 2008. Results. The age of the patients ranged from 1 month to 19years old (average age: 6.6). 17 were male and 16 female. The surgeries in which we used, by different reasons, human pericardium for dural closure were: 7 posterior fossa tumors, 6supratentorial tumors, 5 traumatic brain injury (intracranial hypertension), 3 Arnold-Chiari malformations, 3 spontaneous haematomas, 2 arterio-venous malformations, 2 lumbo-sacrallipomas, 2 encephaloceles, 1 surgery for epilepsy, 1 spinal cord tumor, and 1 brainstem tumor. The complications found were: 2 pseudomeningoceles and 1 CSF fistula. There were no systemicallergic reactions or local skin changes or infections. Follow up: 18.6 months.Conclusion. We considered that human pericardium is an excellent, effective and safe cranial and spinal dural substitute, presenting advantages such as: to be impermeable, easy tosuture and to handle, providing hermetic sealing, low antigenicidadand not to induce reactions to strange body.

Aneurismas experimentales en ratas / Experimental aneurysms in rats

Objective. 1. The creation of an aneurysm model in an arterial bifurcation for microsurgical training in rats. 2. To verify angiographically the aneurysms patency performing endovascular maneuvers. Material and method. 10 Wistar rats weighted 400-600g were used. Ten aneurysms were performed, 9 in the final aortic bifurcation and one in the origin of the renal artery. The aneurismal sac derived from the external iliac vein. Angiography in each animal was done to examine aneurysm patency. At the same time we tried to manipulate microcateter and microguidewire in the aortic lumen. After that the aneurysm was microscopically inspected in order to verify the angiographic findings. Results. The aneurysms and the angiographic study could be performed in every animal. Four aortic and the renal artery aneurysms were not visualized in the angiography (totally thrombosed). The other five were partially thrombosed. Under microscopic aneurismal inspection could be found thrombus into the sac. The endovascular navigation was difficult due to the animal size. Conclusion. The bifurcation aneurysm model is a good microsurgical training. In our hands the aneurysms created had a high rate of spontaneous thrombosis. Because of animal size it is not a good model for endovascular training.

Hematomas intraparenquimatosos sin causa aparente en pediatría / Intraparenchimatous hematomas without apparent etiology in pediatrics

Objetive: spontaneous intracerebral hemorrhage (ICH) in pediatric population has many causes. Some patients remain non diagnosed in spite of being fully checked. Our objective is to analyze those patients whose etiology could not be found. Material and method: from march 2006 to april 2009 we studied 39 patients with ICH. Trauma and coagulation disorders were excluded. CT scan was performed to diagnose the bleeding. MRI and al least two digital subtraction angiography (DSA) were the methods of diagnosis. Results: thirty-nine presented ICH. Twenty presented AVM, five aneurysm, one tumor, three dural sinus thrombosis. In 4 females and 6 male (age 2 months to 12 years) no etiology could be found. Six patients had headache, four sensory loss and two seizures. The cerebellar hemisphere was involved in two patients, basal ganglia in two, thalamus in one and five were subcortical located. Six presented intraventricular hemorrhage , and needed extremal ventricular drainage. Six patients underwent clot removal because of mass effect. All patients survived. Four remained with hemiparesia. Conclusion: in 25% of patients with ICH no etiology could be found in spite of being fully checked. The majority of them were between 6 a12 years old. There were no children over 12 years. There were no deaths in this serie; therefore early surgery due to increased ICP seems to have a favorable outcome.

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female – mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.

Ependimoma intracraneano en la infancia / Intracranial ependymoma in infancy and childhood

El ependimoma es un tumor de lento crecimiento que se origina de células de la pared de los ventrículos cerebrales o del canal ependimario, que afecta preferentemente a los niños y adultos jóvenes. Corresponde histológicamente a grado II de la clasificación de la OMS (Organización Mundial de la Salud). La variante anaplásica corresponde al grado III. Representa el 6.12 % de los tumores intracraneanos en la infancia. La edad más frecuente es alrededor de los 6 años y no hay prevalencia de sexos. El ependimoma puede nacer en cualquier punto del sistema ventricular o del canal espinal o, alejados de ellos, de remanentes aislados en el parénquima cerebral. En los niños ocupa el tercer lugar de frecuencia en la fosa posterior, después del meduloblastoma y astrocitoma. La conducta biológica del ependimoma varía totalmente según esté localizado en el compartimiento supratentorial o en la fosa posterior: el ependimoma supratentorial no tiene un patrón radiológico característico y suele ser anaplásico; no obstante, como la resección es posible en la mayoría de los casos se puede hablar de curación. En cambio el ependimoma de fosa posterior que tiene un patrón radiológico característico, generalmente nace del piso del cuarto ventrículo lo que impide la resección total y por ende la curación, aunque sea de bajo grado. El tratamiento de elección del ependimoma es la resección total, y cuando ello no es posible se completa el tratamiento con radioterapia local conformada (acelerador lineal) sea un ependimoma de alto o bajo grado. La quimioterapia no es efectiva en el ependimoma.

Ependymoma is a slowly growing tumor, that takes origin from cells of the ventricular wall or the epndymal canal. It affects preferentially children and jung adults. Histologically it belongs to the OMS type II classification. The anaplastic variant belongs to the OMS grade III. It represents the 6.12% of the intracranial tumors in infancy andthe most frequent age of appearance is around 6 years without sex preference.In children is the third most frequent tumor of the posterior fossa, after medulloblastoma and astrocytoma.The biological behaviour of ependymoma varies after its locationin the posterior fossa or the supratentorial room. The supratentorialependymomoma has not a characteristic radiological pattern and is frequently anaplastic; however, because the totalresection is possible, it can be cured in most cases. On the other hand, the posterior fossa ependymoma having a typical radiologic pattern, grows habitually from the floor of the fourthventricle making impossible the total resection and therefore curation, even in low grade tumors. The elective treatment of ependymoma is total resection and, if not possible, it can be complemented with conformed localradiotherapy with linear acclerator, both for low or high grade tumors. Chemotherapy is ineffective in ependymomas.

Heridas por arma de fuego en pacientes pediátricos / Gun-shot wounds in pediatric patients

Objective. To describe and analyze a series of pediatric patients who underwent surgery for craniocerebral gunshot wounds. Material and method. The study is a retrospective review of the clinical charts of patients with cranial gunshot injuries seen between 1987 and 2007. Results. In this period 40 patients were admitted and 38 were operated on always with general anesthesia. Mean age of the children was 7 years. Twenty eight patients were male and twelve female. Twelve children sustained transhemipheric injurieds, ten extradural injuries, 9 tangencial injuries, 5 bihemipheric injuries and 3 transventricular injuries. Mortality rate was 20% with a mean follow up of 36 months. Conclusion. We conclude that almost all cranial gunshot patients should initially receive aggressive surgical therapy with a previous CT scan.

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.

Neuroendoscopia guiada por neuronavegación / Neuroendoscopy guided by neuronavigation

Objetivo. Describir y analizar una serie de pacientes en los que se utilizó neuroendoscopía guiada por neuronavegación para la resolución de diferentes patologías neuroquirúrgicas. Material y método. Se realizó una análisis retrospectivo de las historias clínicas de los pacientes en los que se combinaron la utilización del neuroendoscopio de óptica rígida de 0° o 30° STORZ-RECQ y de los neuronavegadores (BRAIN LAB Vectorvisión II ó Electra). Resultados. Desde abril de 2001 hasta diciembre de 2006, operamos 14 pacientes combinando ambas tecnologías. Las edades de los pacientes fueron de entre 10 meses y 10 años (promedio 7.5 años). Las patologías que motivaron la cirugía fueron: seis quistes aracnoideos próximos a ó dentro de ventrículos pequeños, cuatro tumores hipotalámicos, tres ventrículos excluidos y una cavidad porencefálica. La precisión del procedimiento de neuronavegación fue siempre inferiora 1mm, sin embargo no pudimos acceder al sistema ventricular en uno de los catorce pacientes. En los trece restantes se logró el planeamiento prequirúrgico en forma adecuada. Conclusión. La neuroendoscopia guiada por estereotaxia sin marco con neuronavegador es una técnica precisa y segura cuando la patología es solucionable endoscópicamente y los ventrículos son pequeños.

Malformación de la vena de Galeno / Vein of Galen malformation

Objetivo: describir y analizar la embriología, anatomía, angioarquitectura y clínica de la malformación de la vena de Galeno. Método: revisión bibliográfica con actualización del tema. Resultados: la malformación de la vena de Galeno es una anormalidad congénita que presenta el 30% de las lesiones vasculares en los pacientes pediátricos. Se caracteriza por la persistencia de una vena embrionaria: la vena prosencefálica media, asociada a shunts arteriales por persistencia de una conexión fistulosa con arterias coroideas primitivas. La vena de Galeno se localiza en la región pineal, dentro de la cisterna cuadrigeminal. Se forma por la unión de las venas cerebrales internas y cursa posteriormente drenando hacia el seno recto. Su longitud varía de 3,1 a 25 mm. Las malformaciones de la vena de Galeno se clasifican en 1)malformación aneurismática verdadera y 2) dilatación aneurismática. Basándose en la angioarquitectura el primer tipo se divide en 2 formas: mural y coroidea. La presentación clínica es variada y depende del tipo. El diagnóstico prenatal es posible con la ecografía y la resonancia magnética. El diagnóstico definitivo se logra con la angiografía digital. El éxito del tratamiento depende del reconocimiento de sus tipos y formas y, de un adecuado equipo de especialistas. Conclusión: el conocimiento de la embriología, anatomía y, tipos y formas de la malformación de la vena de Galeno, aseguran el éxito del diagnóstico y tratamiento. Palabras clave: angioarquitectura, clasificación, malformación de la vena de Galeno

Objective: to describe and analyse the embryology, anatomy, angioarchitecture and clinical presentation of the vein of Galen malformation. Method: biblographic review up to date. Results: the vein of Galen malformation is a congenital anomaly that represents 30% of all the vascular lesions in the pediatric population. It is secondary to the persistence of an embrionary vein: the medial prosencephalic vein, associated with arterial shunts secondary to the persistence of a fistulous connection with the coroideal primitive arteries. The vein of Galen is localized at the pineal region, within the cuadrigeminal cistern. It is formed by the union of both internal cerebral veins and drains posteriorly into strait sinus. it has a length of 3.1 to 25 mm. They are classified in: 1) truth aneurismal malformation and 2) aneurismal dilatation. Based on its angioarchitecture the first type is divided into 2 forms: mural and coroidal. The clinical presentation is variable and depends on the type. Prenatal diagnosis is possible with ecography and magnetic resonance. Definitive diagnosis is performed with digital angiography. A successful treatment depends on the recognition of the different types and forms and on the presence of an appropriate team of specialists. Conclusion: the knowledge of the embryology, anatomyand types and forms of the vein of Galen malformation assure the success of its diagnosis and treatment. Key words: angioarchitecture, classification, vein of Galen malformation.

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.

Diferentes tecnicas neuroendoscopicas para el tratamiento de la hidrocefalia en pediatria. Estudio multicentrico en 360 pacientes / Different neuroendoscopical Techniques for the treatment of pediatric hydrocephalus Study in 360 patients

Objective: To analize our experience in the endoscopical treatment of pediatrichydrocephalus. Methods: In a retrospective study we analyzed 360 hidrocephalic patients treated endoscopically in 5 Pediatric Neurosurgical Services in Buenos Aires. 374 procedures were performed: 207 third-ventriculostomies, 70 septal fenestrations, 48 catheter implantations, 26 cyst-ventriculostomies, 11 septomies, 7 catheter removals, 4 aqueductoplasties and 1 monoplasty. Results: Third ventriculostomy was the most frequent procedure with a very low failure rate (19,8 por ciento). The overall complication rate was 4,5 por ciento. IN pineal tumor related hydrocephalus, a simultaneous biopsy procedure was always sucessful. Conclusions: Endoscopic treatment of hydrocephalus must always be consideraded as an effective method and as the first choice treatment in an important number of patients