[Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge]. / Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.

INTRODUCTION: Trisomy 9 is an unusual chromosome abnormality in live-born patients, which is frequently accompanied by functional and structural anomalies of the central nervous system. Among many other alterations, several studies have been published in the English-speaking literature that show an association between chromosome 9 abnormality and pathologies affecting the choroid plexuses. CASE REPORT: We report the case of a 4-month-old male with trisomy 9 mosaicism associated to hydrocephalus secondary to choroid plexus hyperplasia, who was referred due to a clinical picture of intracranial hypertension. The cerebrospinal fluid (CSF) drainage procedure that was initially chosen caused massive ascites due to an excessive production of CSF, and led to a cascade of multiple surgical interventions, which included endoscopic and drainage procedures. CONCLUSIONS: This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices.

TITLE: Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomia 9. Un verdadero reto diagnostico y terapeutico.Introduccion. La trisomia 9 es una cromosomopatia inusual en pacientes nacidos vivos, que frecuentemente se acompaña de anomalias funcionales y estructurales del sistema nervioso central. Entre otras muchas alteraciones, varios trabajos en la bibliografia anglosajona demuestran una asociacion entre cromosomopatia 9 y patologia de los plexos coroideos. Caso clinico. Varon de 4 meses de vida con mosaicismo de trisomia 9 asociado a hidrocefalia secundaria a hiperplasia de los plexos coroideos, que fue remitido por clinica de hipertension intracraneal. El procedimiento derivativo de liquido cefalorraquideo por el que optamos inicialmente provoco una ascitis masiva debida a la produccion desmesurada de liquido cefalorraquideo, y desemboco en una cascada de multiples intervenciones quirurgicas, entre las que se incluyeron procedimientos endoscopicos y derivativos. Conclusiones. Se trata de un ejemplo mas de asociacion entre patologia de los plexos coroideos y cromosomopatia 9. Debido a su escasa incidencia, es dificil establecer el diagnostico de hidrocefalia secundaria a hiperplasia de los plexos y, por tanto, el tratamiento mas adecuado. En este tipo de hidrocefalia existe un doble mecanismo fisiopatologico, que implica un aumento de produccion de liquido cefalorraquideo y una disminucion de su reabsorcion. A pesar de tener en cuenta dicha consideracion, el tratamiento de la hidrocefalia secundaria a hiperplasia de los plexos supone un verdadero reto que habitualmente pasa por multiples procedimientos quirurgicos, desde la plexectomia o coagulacion de los plexos coroideos hasta la implantacion de dispositivos de derivacion de liquido cefalorraquideo.

Papel de la craniectomía descompresiva en el tratamiento de la hipertensión intracraneal refractaria, secundaria a encefalitis / The role of decompressive craniectomy in management of refractory intracranial hypertension due to encephalitis

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[Meduloblastoma: infrequent onset in local recurrence]. / Meduloblastoma: una presentación infrecuente en la recurrencia local.

INTRODUCTION. Meduloblastoma is high potential to recur and spread tumour. CASE REPORT. One case posterior fosse meduloblas toma in six years old boy who received 3 surgeries, chemotherapy and radiotherapy, with local recurrence and extracranial extension into neck laterocervical musculature. CONCLUSION. Although meduloblastoma extraneural metastases are not isolated phenomenon, meduloblastoma extracraneal extension is uncommon phenomenon in local recurrence.

Meduloblastoma: una presentación infrecuente en la recurrencia local / Meduloblastoma: infrequent onset in local recurrence

Introducción. El meduloblastoma es un tumor con un elevado potencial de recurrir y de metastatizar.Caso clínico. Presentación de un caso de meduloblastoma de fosa posterior en un niño de 6 años intervenido en 3 ocasiones y tratado con quimioterapia y radioterapia con una recurrencia local que se extendía extracranealmente a nivel de la musculatura laterocervical del cuello.Conclusión. Aunque las metástasis extracraneales del meduloblastoma no es un fenómeno aislado, la extensión extracraneal del meduloblastoma es un fenó-meno poco frecuente en la recurrencia local (AU)

Introduction. Meduloblastoma is high potential to recur and spread tumour.Case report. One case posterior fosse meduloblastoma in six years old boy who received 3 surgeries, chemotherapy and radiotherapy, with local recurrence and extracranial extension into neck laterocervical musculature.Conclusion. Although meduloblastoma extraneural metastases are not isolated phenomenon, meduloblastoma extracraneal extension is uncommon phenomenon in local recurrence (AU)

[Occult anterior sacral meningocele]. / Meningocele sacro anterior oculto.

BACKGROUND: The anterior sacral meningocele is a rare form of spinal dysraphism located in the presacral extraperitoneal space. It is generally asymptomatic and appears as a small pelvic mass. CASE DESCRIPTION: We present a case of a 10 year-old male that develops a bacteria meningitis because of a pararectal abscess that is connected with an anterior sacral meningocele. We successfully treated it using a posterior approach. CONCLUSION: The aim of the surgery is to repair the dural defect and to obliterate the communication between the thecal sac and the herniation defect. The posterior approach is the simplest and most effective surgical technique because the lower risk of neurological injury than the anterior approach.

Meningocele sacro anterior oculto / Occult anterior sacral meningocele

Introducción. El meningocele sacro anterior es una rara forma de disrafismo espinal, localizado en el espacio presacro extraperitoneal. Generalmente es asintomático y suele presentarse como una pequeña masa pélvica. Caso clínico. Presentamos el caso de un niño de 10 años de edad que desarrolla una meningitis bacteriana consecuencia de un absceso pararectal que se comunica con una cavidad meningocélica sacra anterior. Recibe tratamiento quirúrgico realizándose con éxito un abor­daje posterior. Conclusión. La finalidad de la cirugía es reparar el defecto dural y obliterar la comunicación existente entre el saco tecal y el defecto herniario. El abordaje posterior es la técnica quirúrgica más efectiva puesto que tiene menor riesgo de daño neurológico que el abor­daje anterior (AU)

Background. The anterior sacral meningocele is a rare form of spinal dysraphism located in the presacral extraperitoneal space. It is generally asymptomatic and appears as a small pelvic mass. Case description. We present a case of a 10 year-old male that developes a bacteria meningitis because of a pararectal abscess that is connected with an anterior sacral meningocele. We successfully treated it using a posterior approach. Conclusion. The aim of the surgery is to repair the dural defect and to obliterate the communication between the thecal sac and the herniation defect. The posterior approach is the simplest and most effective surgical technique because the lower risk of neurologi­cal injury than the anterior approach (AU)

[Pediatric intensive care after brain tumor surgery]. / Postoperatorio de tumores cerebrales en la unidad de cuidados intensivos pediátricos.

INTRODUCTION: Primary brain tumors are the most common solid tumors in children. Surgery is the basis of treatment for these patients, who require postoperative admission to the ICU-P. The aim of this study was to at the epidemiology of brain tumors of patients admitted to our ICU-P and to analyze the progress of these children in the postoperative period. PATIENTS AND METHODS: Retrospective-prospective study of children admitted to our unit after brain tumor surgery between January 1998 and January 2007. We collected information such as, personal details, clinical characteristics, type of intervention and postoperative period. RESULTS: We reviewed 161 postoperative periods, corresponding to 134 patients (54.5% male). The mean age was 7 years and 8 months +/- 5 months. The most common location was the posterior fossa (44.8%). The most common histological type was low grade/intermediate astrocytoma (44.7%). The most frequent complication was diabetes insipidus (9.9%). Resection was complete in 58.4% patients. The median stay in the ICU-P was 2 days (0-61 days). 3 patients died after surgery. CONCLUSIONS: The epidemiology of the group of patients admitted to our ICU-P is similar to the general population. The most common complication is diabetes insipidus.

Postoperatorio de tumores cerebrales en la unidad de cuidados intensivos pediátricos / Pediatric intensive care after brain tumor surgery

Introducción: Los tumores primarios cerebrales son la neoplasia sólida más frecuente en la infancia. La base fundamental del tratamiento de estos pacientes es la cirugía, cuyo postoperatorio requiere ingreso en la unidad de cuidados intensivos pediátricos (UCIP). Los objetivos de nuestro trabajo son: conocer la epidemiología de los tumores que ingresan en nuestra unidad y analizar el postoperatorio de estos niños. Pacientes y métodos: Estudio prospectivo y retrospectivo de todos los niños intervenidos de tumor cerebral desde enero de 1998 hasta enero de 2007 en nuestro hospital. Se recogen datos referentes a filiación, características clínicas, tipo de intervención y postoperatorio. Resultados: Se revisan 161 postoperatorios, correspondientes a 134 pacientes (el 54,5%, varones). La media ± desviación estándar de edad es 7 años y 8 meses ± 5 meses. La localización más frecuente es la fosa posterior (44,8%). El tipo histológico principal es el astrocitoma de grado bajo/intermedio (44,7%). La complicación más frecuente es la diabetes insípida (9,9%). La resección es total en el 58,4% de los pacientes. La mediana de estancia en UCIP es de 2 días (0-61 días). Fallecieron 3 pacientes tras la intervención. Conclusiones: La epidemiología del grupo de pacientes que llega a UCIP es similar a la población general. La complicación más frecuente es la diabetes insípida (AU)

Introduction: Primary brain tumors are the most common solid tumors in children. Surgery is the basis of treatment for these patients, who require postoperative admission to the ICU-P. The aim of this study was to at the epidemiology of brain tumors of patients admitted to our ICU-P and to analyze the progress of these children in the postoperative period. Patients and methods: Retrospective-prospective study of children admitted to our unit after brain tumor surgery between January 1998 and January 2007. We collected information such as, personal details, clinical characteristics, type of intervention and postoperative period. Results: We reviewed 161 postoperative periods, corresponding to 134 patients (54.5% male). The mean age was 7 years and 8 months±5 months. The most common location was the posterior fossa (44.8%). The most common histological type was low grade/intermediate astrocytoma (44.7%). The most frequent complication was diabetes insipidus (9.9%). Resection was complete in 58.4% patients. The median stay in the ICU-P was 2 days (0–61 days). 3 patients died after surgery .Conclusions: The epidemiology of the group of patients admitted to our ICU-P is similar to the general population. The most common complication is diabetes insipidus (AU)

[Intracranial tuberculoma: a case report and review of the literature]. / Tuberculoma intracraneal: caso clínico y revisión de la bibliografía.

INTRODUCTION: Cerebral tuberculoma constitutes an important neurotuberculosis clinical form with high morbimortality and important diagnoses difficulty. CASE REPORT: Only giant cerebral tuberculoma case in 3 years-old man who present dizziness and surgically treated by diagnostic suspicion of cerebral tumor. CONCLUSION: Cerebral tuberculomas can often be confused with cerebral tumors when they are not included in differential diagnosis.

Tuberculoma intracraneal: caso clínico y revisión de la bibliografía / Intracranial tuberculoma: A case report and review of the literature

Introducción. El tuberculoma cerebral constituye una forma clínica importante de neurotuberculosis con una elevada morbimortalidad y una notable dificultad diagnóstica. Caso clínico. Único caso de tuberculoma cerebral gigante en un niño de 3 años, que comenzó con crisis epiléptica y se trató quirúrgicamente por sospecha diagnóstica de tumor cerebral. Conclusión. Los tuberculomas cerebrales pueden confundirse muchas veces con tumores cerebrales cuando no se incluyen enel diagnóstico diferencial

Introduction. Cerebral tuberculoma constitutes an important neurotuberculosis clinical form with high morbimortality and important diagnoses difficulty. Case report. Only giant cerebral tuberculoma case in 3 years-old man who present dizziness and surgically treated by diagnostic suspicion of cerebral tumor. Conclusion. Cerebral tuberculomas can often be confused with cerebral tumors when they are not included in differential diagnosis

[Sinus pericranii. A case report]. / Sinus pericranii. A propósito de un caso.

INTRODUCTION: Sinus pericranii is an unusual vascular anomaly characterised by communication between diploic and epicranial veins and a dural sinus. It is therefore an abnormal communication between the intracranial and extracranial circulatory systems. CASE REPORT: We report a case of sinus pericranii in an 8-month-old boy, with typical clinical and radiological manifestations. CONCLUSIONS: Sinus pericranii is a rare condition in paediatric pathology, which is generally asymptomatic and is usually found in the area of the anterior fontanelle. The fundamental diagnostic method is magnetic resonance imaging of the brain with and without contrast; surgery is the preferred treatment, although rare cases of spontaneous thrombosis have been reported.

Sinus pericranii. A propósito de un caso / Sinus pericranii, A case report

Introducción. El sinus pericranii es una anomalía vascular poco frecuente consistente en una comunicación entre un seno dural y las venas diploicas y epicraneales. Es pues una comunicación anómala entre la circulación intra y extracraneal.Caso clínico. Presentamos un caso de sinus pericranii en un niño de 8 meses, con unas típicas manifestaciones clínicas y radiológicas. Conclusiones. El sinus pericranii es una entidad poco frecuente en la patología pediátrica, que cursa generalmente asintomática, cuya localización suele ser en el sector de la fontanela anterior. El método diagnóstico fundamental es la resonancia magnética cerebral con y sin contraste, siendo su tratamiento de elección el quirúrgico, aunque se ha descrito enraros casos su trombosis espontanea

Introduction. Sinus pericranii is an unusual vascular anomaly characterised by communication between diploicand epicranial veins and a dural sinus. It is therefore an abnormal communication between the intracranial and extracranial circulatory systems. Case report. We report a case of sinus pericranii in an 8-month-old boy, with typical clinical and radiological manifestations. Conclusions. Sinus pericranii is a rare condition in paediatric pathology, which is generally asymptomatic and is usually found in the area of the anterior fontanelle. The fundamental diagnostic method is magneticresonance imaging of the brain with and without contrast; surgery is the preferred treatment, although rare cases of spontaneous thrombosis have been reported

[Mediastinal lipoblastoma with intramedullary extension]. / Lipoblastoma mediastínico con extensión intramedular.

INTRODUCTION: Lipoblastomatous lesions are mesenchymal tumors of fetal-embryonal fat that are classified into two types: a superficial, well-defined mass (lipoblastoma) or an infiltrative lesion (lipoblastomatosis). CASE REPORT: An unique case of mediastinal lipoblastoma in a 27-month-old boy with spinal epidural extension from C7-D9, clinically manifested as a progressive painful. CONCLUSION: The treatment of choice is the complete excision, with high risk of recurrence, suggesting a periodical clinical evaluation with neuroimaging studies.

[Multiple familial cerebral cavernomatosis]. / Cavernomatosis múltiple cerebral familiar.

INTRODUCTION: Intracraneal vascular malformations are congenital lesions due to alterations in the development of arteriocapillary network. Cavernomas are present in 0.4% of people, and represent 5-13% of all cerebrovascular malformations. They are multilobulated clearly delimited lesions that contain blood at different evolution. Cavernomas can be single or multiple, and sporadic or familial. Inheritance pattern in familial cases is autosomal dominant and three involved genes have been described. CASE REPORTS: We made a retrospective review of clinical histories of two patients diagnosed of multiple familial cavernomatosis. First patient's onset was with partial seizures. Magnetic resonance (MR) showed a frontal cavernoma probable responsible of seizures, and other lesions in frontal and parietal lobes. Second patient consulted for psychomotor delay and behaviour disorder. MR showed multiple cavernomas. In the first patient, one lesion was surgically removed. In second patient, the attitude was expectant. In both cases familial study was done and multiple cavernomas were found in both parents. CONCLUSIONS: Cavernomas are a type of vascular malformations with specific histological features. Usual clinical characteristics are seizures and parenchymatous bleeding. The appearance of MR has permitted the diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for this purpose. In familial cases, multiple lesions are found in 84%, often in association with family history of seizures. Surgical treatment must be considered in patients with symptomatic or progressive lesions that are accessible. All cases must be clinically and MR followed.

Cavernomatosis múltiple cerebral familiar / Multiple familial cerebral cavernomatosis

Introducción. Las malformaciones vasculares intracraneales son lesiones congénitas debidas a alteraciones en el desarrollo de la red arteriolocapilar. Los angiomas cavernosos están presentes en el 0,4% de los individuos y constituyen el 5- 13% de las malformaciones vasculares. Son lesiones multilobuladas, bien delimitadas, con sangre en distintos estadios evolutivos. Pueden ser únicos o múltiples, esporádicos o familiares. En los casos familiares, la herencia es autosómica dominante. Se han descrito hasta ahora tres genes implicados. Casos clínicos. Se presentan dos niños diagnosticados de cavernomatosis múltiple familiar. Uno consultó por crisis parciales y se objetivó un angioma cavernoso frontal derecho, probablemente responsable de las crisis, y otros dos en el frontal y el parietal izquierdo. El otro niño consultó por un retraso psicomotor y trastorno de conducta, y se observaron múltiples angiomas cavernosos diseminados. En el primer caso se realizó una exéresis de una de las lesiones, mientras que en el segundo se decidió una actitud conservadora. Se realizó un estudio familiar y se encontraron angiomas cavernosos múltiples en ambos padres. Conclusiones. Los angiomas cavernosos son un tipo de malformación vascular con unas características histológicas determinadas. Los síntomas clínicos habituales son convulsiones y hemorragia parenquimatosa. La aparición de la resonancia magnética ha permitido el diagnóstico de angiomas cavernosos asintomáticos, y actualmente se considera la técnica de elección para el diagnóstico. La forma familiar presenta lesiones múltiples hasta en un 84% y se asocia frecuentemente con una historia familiar de crisis epilépticas. El tratamiento quirúrgico debe plantearse en casos de lesiones de localización accesible que hayan producido síntomas en repetidas ocasiones o de forma progresiva. En todos los casos se realizarán controles clínicos y de resonancia magnética

Introduction. Intracraneal vascular malformations are congenital lesions due to alterations in the development of arteriocapillary network. Cavernomas are present in 0,4% of people, and represent 5-13% of all cerebrovascular malformations. They are multilobulated clearly delimited lesions that contain blood at different evolution. Cavernomas can be single or multiple, and sporadic or familial. Inheritance pattern in familial cases is autosomal dominant and three involved genes have been described. Case reports. We made a retrospective review of clinical histories of two patients diagnosed of multiple familial cavernomatosis. First patient’s onset was with partial seizures. Magnetic resonance (MR) showed a frontal cavernoma probable responsible of seizures, and other lesions in frontal and parietal lobes. Second patient consulted for psychomotor delay and behaviour disorder. MR showed multiple cavernomas. In the first patient, one lesion was surgically removed. In second patient, the attitude was expectant. In both cases familial study was done and multiple cavernomas were found in both parents. Conclusions. Cavernomas are a type of vascular malformations with specific histological features. Usual clinical characteristics are seizures and parenchymatous bleeding. The appearance of MR has permitted the diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for this purpose. In familial cases, multiple lesions are found in 84%, often in association with family history of seizures. Surgical treatment must be considered in patients with symptomatic or progressive lesions that are accessible. All cases must be clinically and MR followed

Lipoblastoma mediastínico con extensión intramedular / Mediastinal lipoblastoma with intramedullary extension

Introducción. Las lesiones lipoblastomatosas son tumores mesenquimales grasos fetoembrionarios que se clasifican en dos tipos, uno superficial como masa bien definida (lipoblastoma) y otro infiltrativo (lipoblastomatosis). Caso clínico. Caso único de lipoblastoma mediastínico en un niño de 27 meses de edad con una extensión epidural intramedular desde C7 a D9, clínicamente manifestado por un dolor progresivo. Conclusión. El tratamiento de elección es el quirúrgico, aunque el porcentaje de recidivas es alto, por lo que se aconseja control seriado con resonancia magnética para el diagnóstico precoz de éstas

Introduction. Lipoblastomatous lesions are mesenchymal tumors of fetal-embryonal fat that are classified into two types: a superficial, well-defined mass (lipoblastoma) or an infiltrative lesion (lipoblastomatosis). Case report. An unique case of mediastinal lipoblastoma in a 27-month-old boy with spinal epidural extension from C7-D9, clinically manifested as a progressive painful. Conclusion. The treatment of choice is the complete excision, with high risk of recurrence, suggesting a periodical clinical evaluation with neuroimaging studies