Doble derivación ventrículo y cisto biliar en el tratamiento de quiste aracnoideo gigante supraselar con hidrocefalia secundaria / Double ventriculo-gallbladder shunt in the treatment of giant suprasellar arachnoid cyst with secondary hydrocephalus

La derivación ventrículo biliar se describe como un procedimiento alternativo eficaz en el tratamiento de la hidrocefalia. Presentamos el caso de un paciente de 19 años de edad diagnosticado de quiste aracnoideo supraselar e hidrocefalia, portador de doble sistema valvular de derivación ventrículo peritoneal y cisto peritoneal desde la infancia. Tras varios recambios por fallo peritoneal, fue sometido a derivación ventriculoauricular, con complicaciones asociadas y posterior recolocación a peritoneo. Tras nueva disfunción valvular por complicaciones peritoneales, se planteó la derivación ventriculobiliar como tratamiento alternativo para este paciente, que resultó segura y eficaz en la resolución de la clínica del paciente, siendo dado de alta, manteniendo estabilidad clínica durante más de 2 años de seguimiento. (AU)

Ventriculo-gallbladder shunt is described as an effective alternative procedure in the treatment of hydrocephalus. We present the case of a 19-year-old patient diagnosed with suprasellar arachnoid cyst since his childhood and hydrocephalus, with a double shunt; ventriculoperitoneal and peritoneal cyst shunt. After several replacements due to peritoneal failure, he underwent ventriculoatrial shunt, with associated complications and subsequent repositioning to the peritoneum. After new valve dysfunction due to peritoneal complications, ventriculo-gallbladder shunt was proposed as an alternative treatment for this patient, which was safe and effective in resolving the patient's symptoms, and the patient was discharged, maintaining clinical stability in the follow-up more than 2 years later. (AU)

Double ventriculo-gallbladder shunt in the treatment of giant suprasellar arachnoid cyst with secondary hydrocephalus.

Ventriculo-gallbladder shunt is described as an effective alternative procedure in the treatment of hydrocephalus. We present the case of a 19-year-old patient diagnosed with suprasellar arachnoid cyst since his childhood and hydrocephalus, with a double shunt; ventriculo peritoneal and peritoneal cyst shunt. After several replacements due to peritoneal failure, he underwent ventriculoatrial shunt, with associated complications and subsequent repositioning to the peritoneum. After new valve dysfunction due to peritoneal complications, ventriculo-gallbladder shunt was proposed as an alternative treatment for this patient, which was safe and effective in resolving the patient's symptoms, and the patient was discharged, maintaining clinical stability in the follow-up more than 2 years later.

Quiste aracnoideo (Galassi Tipo II) y síntomas neuropsiquiátricos: a propósito de un caso / Arachnoid Cyst (Galassi Type II) and neuropsychiatry symptoms: about a case

INTRODUCCIÓN: un quiste aracnoideo es originado de la alteración de una de las membranas de la meninges (aracnoides), de predominio en la fosa craneal media; es una patología poco común que cause síntomas y si ocurren, se pueden presentar manifestaciones neuropsiquiatrías. OBJETIVO: exponer los datos clínicos; y la metodología diagnóstica y terapéutica. CASO: presentamos una paciente femenina de 24 años de edad; sin antecedentes personales médicos psiquiátricos y médicos no psiquiátricos conocidos, quien presenta una historia con un mes de evolución de síntomas psicóticos y cambios conductuales. Se le realizo CAT cerebral simple y luego una resonancia magnética cerebral contrastada para definir el tamaño del quiste aracnoideo, por los posibles síntomas neuropsiquiátricos encontrados. Por medio de exámenes de laboratorios, estudios electrofisiológicos (electroencefalograma), neuroimágenes y evaluación clínica. Se decide presentar las características clínicas encontradas de la paciente quien requirió manejo con antipsicóticos, benzodiacepinas y estabilizador del humor con gradual mejoría de sus sintomatologías de ingreso (agitación psicomotora y psicosis).

INTRODUCTION: an arachnoid cyst is caused by the alteration of one of the membranes of the meninges (arachnoid), predominantly in the middle cranial fossa; It is an uncommon pathology that causes symptoms and if they occur, neuropsychiatric manifestations may take place. OBJECTIVE: expose the clinical data; and the diagnostic and therapeutic methodology. CASE: we present a 24-year-old female patient; with no personal history of psychiatric and known non-psychiatric medical records, whom presents a story with a month of evolution of psychotic symptoms and behavioral changes. A simple cerebral CAT was performed and then a cerebral magnetic resonance imaging with contrast to define the size of the arachnoid cyst, due to the possible neuropsychiatric symptoms found. Through laboratory tests, electrophysiological studies (electroencephalogram), neuroimaging and clinical evaluation. It was decided to present the clinical characteristics of the patient who required management with antipsychotics, benzodiazepines and mood stabilizer with gradual improvement of her admission symptoms (psychomotor agitation and psychosis)

Haberland syndrome with bilateral ocular involvement. / Síndrome de Haberland con afectación ocular bilateral.

Haberland syndrome or encephalocutaneous lipomatosis is a very uncommon syndrome that is characterised by changes in the skin, eye, and central nervous system. It was first described in 1970 by Haberland and Perou, with about 60 cases having been reported since then. A case is reported of a 14-week-old male diagnosed with Haberland syndrome with bilateral ocular involvement in the form of palpebral coloboma and choristomas.

Dysembryoplastic neuroepithelial tumor and temporal arachnoid cyst on a child with epilepsy. Which one to operate? Case report and literature review.

A 6 year-old male, with seizures characterized by abnormal epigastric sensation, behavioral arrest, upper extremities search automatisms and secondary tonic-clonic generalization. Magnetic resonance imaging showed a hypointense cystic extra-axial image with an increase in the thickness of the convolutions in the first and second gyri of the right frontal lobe. It was decided to resect the frontal lesion with transoperative motor mapping. Morphological and immunohistochemical findings corresponded to dysembryoplastic neuroepithelial tumor with focal cortical dysplasia. Adequate semiology, analysis of the electroencephalogram, and imaging studies allowed treating adequately the cortical dysplasia. At present, the patient is seizure-free without medication (Engel IA).

Niño de 6 años con crisis caracterizadas por sensación epigástrica, arresto conductual, automatismos de búsqueda y generalización tónico-clónica secundaria. La resonancia magnética mostró una imagen extraaxial quística y un aumento del grosor de las circunvoluciones del primer y segundo giros del lóbulo frontal derecho. Se decidió resecar primero la lesión frontal con guía por mapeo transcortical intraoperatorio. Los hallazgos morfológicos e inmunohistoquímicos mostraron un tumor neuroepitelial disembrioplásico con displasia cortical focal. La semiología, el análisis del electroencefalograma y la imagen permitieron orientar el tratamiento. Actualmente el paciente está libre de crisis y sin medicamentos (Engel IA).

Spinal cord compression secondary to traumatic intradural arachnoid cyst. / Compresión medular secundaria a quiste aracnoideo intradural de etiología traumática.

Spinal arachnoid cysts are rare entities, most of which have a congenital or spontaneous etiology. Secondary cases are infrequent and can constitute a cause of neurological deterioration several months or years after spinal trauma. We present the case of a male patient with traumatic spinal arachnoid cyst, together with a review of the cases published to date. Finally, we explain the different surgical options for this pathology relating them to the physiopathological theories that try to explain the origin of these lesions.

Subdural hygroma secondary to rupture of an intracranial arachnoid cyst: description of 2cases and review of the literature. / Higroma subdural secundario a la rotura de quiste aracnoideo intracraneal: descripción de 2casos y revisión de la literatura.

The appearance of a subdural hygroma after the rupture of an arachnoid cyst wall is extremely rare, with very few cases described in the literature. Most cases are due to a traumatic cause. The therapeutic approach in symptomatic cases is controversial, with a current tendency toward conservative management initially. In those cases that require surgical treatment, multiple therapeutic options are available, with fenestration techniques being recommended as first-line treatment. We describe 2cases treated in our centre and review the literature.

Aracnoidits y quistes aracnoideos espinales múltiples. Revisión bibliográfica a propósito de un caso / ARACNOIDITIS AND MULTIPLE ARACNOID CYSTS : CASE REPORT AND REVISION OF THE LITERATURE

Los quistes aracnoideos son lesiones malformativas que contienen en su interior un líquido de características similares al líquido cefalorraquídeo. Constituyen alrededor del 1% de todos los procesos ocupantes de espacio a nivel intracraneal y, en menor número de casos pueden localizarse a nivel espinal. Presentamos una asociación de aracnoiditis espinal y posterior cavitación.

Arachnoid cysts are malformative lesions that contain a liquid with characteristics similar to cerebrospinal fluid. They constitute about 1% of all space-occupying processes at the intracranial level and, in a smaller number of cases, they can be located at the spinal level. We present an association of spinal arachnoiditis and posterior cavitation

[Spinal extradural arachnoid cyst: A case report and review of literature]. / Quiste aracnoideo extradural espinal: reporte de un caso y revisión de la literatura.

BACKGROUND: Arachnoid cysts of spine are a very rare occurrence. The aetiology still remains unclear, but the most accepted explanation is the existence of areas of weakness in the spinal dura. Symptoms depend on the location in the spine. Magnetic resonance imaging is used for its diagnosis. Management depends of clinical presentation, and the surgery is reserved for patients with neurological impairment. CLINICAL CASE: A case is described of 67 year-old male with myelopathy and radiculopathy symptoms, both diagnosed simultaneously. The magnetic resonance imaging was used to diagnose a thoracolumbar extradural arachnoid cyst from T12-L2 and lumbar spinal canal stenosis. The patient was treated with a puncture procedure to empty the cyst and decompress the neural elements. There was a clinical improvement of myelopathy syndrome after puncture procedure. One month later, the patient underwent a minimally invasive surgical approach to decompress the neural elements in lumbar spine, achieving improvement of the radiculopathy syndrome and neurogenic claudication in both legs. CONCLUSION: There is currently no standard minimally invasive approach to surgically treat these cysts, but if the patient has mild symptoms, clinical observation is recommended.

Lesión como malformación de Chiari secundaria a quiste aracnoidal de la cisterna cuadrigémina / Chiari malformation-like lesion secondary to arachnoid cyst of the quadrigeminal cistern

Se presenta el caso de una mujer de 31 años de edad con cefalea y cervicalgia que se agravaba con la maniobra de Valsalva, presentando además mareos, encontrándose una malformación de Chiari secundario a un quiste aracnoideo cuadrigeminal. Después de efectuada la resonancia magnética diagnóstica, la paciente fue sometida a descompresión del agujero magno y extirpación del quiste cuadrigeminal, seguido por la resolución tanto de la malformación de Chiari y el quiste. Los síntomas desaparecieron después de la cirugía y han permanecido completamente resuelto hasta la actualidad. En pacientes adultos que presentan signos y síntomas de una malformación de Chiari debido a la compresión de la médula por las las amígdalas cerebelosas, la presencia de un quiste aracnoideo de cisterna cuadrigéminal es una rara patología asociada que puede ser tratada quirúrgicamente.

We report a rare case of a 31-year-old woman with headache and pain manifested by cervicalgia that worsened with the Valsalva maneuver and dizziness, who was found to have a Chiari malformation secondary to a posterior fossa arachnoid cyst. After magnetic resonance imagining (MRI), the patient was submitted to foramen magnum decompression and arachnoid cyst removal that were followed by resolution of both the Chiari malformation and the cyst. The symptoms disappeared after surgery and have remained completely resolved to the present day. In adult patients who present with signs and symptoms of Chiari malformation due to direct medulla compression by the tonsils, a quadrigeminal cistern arachnoid cyst is a rare associated pathology that can be treated surgically.

[Microsurgical treatment of intracraneal arachnoid cysts]. / Tratamiento microquirúrgico de los quistes aracnoideos intracraneales.

Craniotomy and fenestration of membranes is one of the main treatment options for symptomatic arachnoid cysts. Open surgery advantages include, direct inspection of the cyst, biopsy sampling, fenestration in multilocular cysts and, in certain locations, cyst communication to basal cisterns. The aim of this paper is to review the advantages and disadvantages of this treatment modality for arachnoid cysts taking into account the different anatomical locations.

Hemifacial spasm caused by a cerebellopontine angle arachnoid cyst. Case report and literature review.

Arachnoid cysts involving the cerebellopontine angle are an unusual cause of hemifacial spasm. The case is reported of a 71-year old woman presenting with a right hemifacial spasm and an ipsilateral arachnoid cyst. Preoperative magnetic resonance imaging findings suggested a neurovascular compression caused by displacement of the facial-acoustic complex and the anterior inferior cerebellar artery by the cyst. Cyst excision and microvascular decompression of the facial nerve achieved permanent relief. The existing cases of arachnoid cysts causing hemifacial spasm are reviewed and the importance of a secondary neurovascular conflict identification and decompression in these cases is highlighted.

[Arachnoid cysts. Historical evolution of the concept and pathophysiological theories]. / Quistes aracnoideos. Evolución histórica del concepto y teorías fisiopatológicas.

Arachnoid cysts of central nervous system are benign collections filled with a liquid that is equal o similar to cerebrospinal fluid, coated with a single layer or flattened arachnoid cells membrane, congenital or secondary to trauma, meningitis or hemorrhage. First described by Bright in 1931, its definition and possible pathophysiology has been debated to date. Our objective is to present a summary of this process.

[Imaging diagnosis of arachnoid cysts]. / Diagnóstico por imagen de los quistes aracnoideos.

Arachnoid cysts are malformed lesions that contain a fluid similar to the cerebrospinal fluid, and are usually located within the arachnoidal membrane. They represent 1% of all intracranial lesions, and in recent years, with the development of radiological techniques, the clinical detectability of arachnoid cysts seems to have increased. Although the majority of diagnosed arachnoid cysts are located in the cranial cavity and especially in the Sylvian fissure, a small number are located at spinal level and they can occur extra- or intra-spinally. An analysis is carried out, detailing the various tests used for the diagnosis of both intracranial and spinal arachnoids cysts, analysing the indications of each one depending on the location of the cysts and patient age.

[«De novo¼ development of a suprasellar arachnoid cyst]. / Desarrollo «de novo¼ de un quiste aracnoideo supraselar.

Intracranial arachnoid cysts are fluid-filled cavities that arise within the cranial arachnoid, representing approximately 1%-2% of all intracranial lesions among the paediatric population. We present the case of a 2-year-old boy who presented with instability and episodes of ocular deviation. A computed tomography scan (CT scan) and magnetic resonance imaging (MRI) of the brain revealed a suprasellar cyst and obstructive hydrocephalus. At birth a transfontanellar ultrasound was normal. The cyst underwent endoscopic fenestration with complete remission of symptoms. In the review of the literature, we found only 6 previous cases of an intracranial arachnoid cyst whose origin was not clearly congenital or traumatic, and ours is the second case of a suprasellar arachnoid cyst to arise de novo. The clinical features, imaging characteristics and treatment of the previously reported cases are discussed.

Estudio neuropsicológico pre y posquirúrgico en dos niños de 8 años de edad con tumor en fosa posterior / Neuropsychological study pre and post surgical in two children aged 8 years with posterior fossa tumor

Objetivo: Identificar y describir las diferencias neuropsicológicas antes y después de resecar el tumor en 2 pacientes de 8 años de edad con una neoplasia en la fosa posterior. Metodología: Se realizó evaluación neuropsicológica pre y posquirúrgica a 2 pacientes de 8 años de edad del Instituto Nacional de Pediatría, uno femenino con quiste aracnoideo en cisterna paravermiana y otro masculino con meduloblastoma en vermis y se compararon los datos obtenidos antes de extirpación de tumor y después de ella. Para la obtención del IQ se aplicó el WISC-IV y para las otras funciones, la Evaluación Neuropsicológica Infantil (ENI), para niños de 5 a 16 años, obteniéndose sus valores en percentiles que se igualaron con la clasificación cualitativa. Resultados: Los 2 pacientes presentaron deficiencias en el IQ, en Funciones Cognitivas, en las Habilidades de Rendimiento Académico, así como en sus Funciones Ejecutivas. A pesar de que no se aplicó quimioterapia ni radioterapia, tanto antes de la cirugía, como después de la extirpación del tumor, empeorando dichas funciones después de la extirpación. Conclusiones: Los tumores en Fosa Posterior originan diversas alteraciones neuropsicológicas similares a las observadas con lesiones en la corteza cerebral, dichas alteraciones se hacen más severas después de la extirpación del tumor; esta evolución puede presentar un dilema a la luz de la Bioética: ¿Se prolonga la vida a expensas de mayor deterioro neurocognitivo al quitar el tumor, o no se opera para evitar mayor deterioro en la calidad de vida y se reduce el tiempo de vida?.

Aim: Identify and describe the neuropsychological differences before and after surgery in 2 patients 8 years of age with a tumor in the posterior fossa. Methodology: Neuropsychological assessment was performed before and after surgery to 2 patients (8 years of age), one female with arachnoid cyst in paravermian cyst and another male with medulloblastoma in vermis and we compared the data obtained before removal of tumor and after. We use the following Neuropsychological Tests: WISC-IV was applied to assess IQ and Child Neuropsychological Assessment (ENI) was to evaluate cognitive functions. Results: The 2 patients had deficits in IQ, cognitive functions, the academic performance skills and his executive skills, even without chemotherapy or radiotherapy, both before surgery and after removal of the tumor. These functions worsened after surgery. Conclusions: Posterior fossa tumors originate many various neuropsychological similar to those observed in cerebral cortex, these changes are most evident after removal of the tumor, this evolution can present a dilemma in light of Bioethics: Is justified to prolong the life at expense of neurocognitive impairment, after removing a big tumor, or not to operate preventing further deterioration in the quality of life and reducing the lifetime?.

[Congenital medulloblastoma associated with intracranial arachnoid cyst]. / Meduloblastoma congénito asociado a quiste aracnoideo intracraneal.

Arachnoid cysts are very common lesions in paediatric patients, with treatment depending on their location and symptomatology. They are usually solitary cysts but may be associated with other central nervous system diseases such as tumours and congenital deformities. We describe the case of a neonate diagnosed with an arachnoid cyst of the quadrigeminal cistern treated by endoscopy. After the operation, the child's condition worsened; a CT scan revealed a midline posterior fossa tumour not visible in the preoperative neuroradiological tests. The tumour, a medulloblastoma, was partially removed. Given the child's age and the poor prognosis, oncological treatment was not undertaken. The association between medulloblastoma and arachnoid cyst is very rare, and we could find only one such case in the literature.

[Intrasellar arachnoid cysts. Two case reports and literature review]. / Quistes aracnoideos intraselares. Presentación de 2casos y revisión bibliográfica.

Intrasellar arachnoid cysts are an uncommon pathology, with only a few cases reported in scientific literature. Their physiopathology is still unknown, with different hypothesis about their development. Differential diagnosis with other cystic sellar lesions is difficult. We report two cases of two intrasellar arachnoid cysts that were treated surgically using transsphenoidal approach and present a review of the literature.

Síndrome del muñeco cabeza de resorte por quiste aracnoideo selar en un adulto / Bobble-head doll syndrome by selar arachnoid cyst in an adult

El síndrome del muñeco cabeza de resorte es un raro trastorno usualmente descrito en la población pediátrica como resultado de lesiones selares y del tercer ventrículo, especialmente quistes coloides. Su fisiopatología no está bien esclarecida. Se ha planteado que los trastornos del funcionamiento de las aferencias y eferencias hacia el núcleo dorso-medial del tálamo originarían los movimientos y estos permanecerían en el tiempo gracias a fenómenos de aprendizaje. Presentamos un raro caso del síndrome del muñeco cabeza de resorte de una paciente adulta como consecuencia de un quiste aracnoideo selar con extensión paraselar a través de la fisura de Silvio. Se realizaron comunicaciones del quiste hacia las cisternas adyacentes con lo cual se logro control completo de los síntomas tras 48 horas del procedimiento.

Bobble head doll syndrome is a rare disorder usually described in the pediatric population as a result of sellar lesions and the third ventricle, especially colloid cysts. Its pathophysiology is not well understood. It has been suggested that functional disorders of the afferent and efferent system to the dorso-medial nucleus of the thalamus causes of the movement and these remain over time through learning phenomena. We present a rare case of bobble head doll syndrome in an adult patient caused by a sellar arachnoid cyst with parasellar extension through the Sylvian fissure. Communications were made from the cyst into adjacent cisterns thereby achieving complete control of symptoms after 48 hours of the procedure.

Meningioma de la vaina del nervio óptico: colaterales optociliares y quiste aracnoideo perióptico / Meningioma of the optic nerve sheath: collateral optociliary veins and the perioptic arachnoidal cyst

En la década sesenta, antes del advenimiento de la tomografía computarizada de las órbitas, los meningiomas de la vaina del nervio óptico eran considerados de rara ocurrencia y su diagnóstico positivo solo era posible en aquellos casos en que por su tamaño producían proptosis ocular, la que conducía a su estirpación quirúrgica. El tumor de crecimiento tubular, comprime el nervio en forma progresiva hasta atrofiarlo por completo. Sus manifestaciones clínicas incluyen la pérdida gradual de la agudeza visual hasta alcanzar la ceguera, edema del nervio óptico y luego atrofia del mismo, y presencia de venas colaterales optociliares. Este complejo signológico se designa como triada de Hoyt-Spencer. Un hecho patológico distintivo de estos tumores objetivado mediante resonancia magnética de órbitas con administración de gadolinio y supresión de grasa, es la presencia de una dilatación quística del espacio subaracnoideo perióptico entre el limite distal del tumor y el polo posterior del ojo. Presentamos el caso de un paciente ilustrativo con demostración histopatológica de las colaterales venosas y del quiste aracnoideo

During the 1970s, before the advent of computerized tomography of orbits, optic nerve sheath meningiomas were considered of rare ocurrence and their positive diagnosis was only posible in those cases in which its size produced ocular proptosis, which led to its surgical removal. Tumor growths in a tubular, compreses progressively the optic nerve and in time produces its atrophic thinning. Clinical manifestation include gradual visual acuity loss until total blindness, optic nerve edema and then optic atrophy, and the presence of collateral optociliary veins. This complex clinical picture is designated as the triad of Hoyt-Spencer. A distinctive fact in these tumors is the presence of a cystic dilatation of the perioptic subarachnoid space between the distal adge of the tumor and the posterior pole, which can be defined using magnetic resonance imaging of the orbits with fat supression following intravenous gadolinium administration. We present the case of a patient with histopatological demostration of the collateral veins and the distal aracnoidal cyst