Rathke's cleft cyst apoplexy in a boy treated by endoscopic endonasal surgery: case report and literature review.

Rathke's cleft cyst (RCC) apoplexy is an uncommon lesion attributed to abnormal vascular supply to the fragile RCC epithelial wall. It is rare in children and very difficult to diagnose without pathologic confirmation. Here, we report an 8-year-old boy who presented with headache and visual deficit. MRI and CT showed a cystic mass in the sellar region. He underwent endoscopic endonasal surgery, and the cystic mass was resected completely via a trans-sphenoidal approach. The lesion was confirmed as RCC apoplexy by intraoperative observation and histopathological examination. Headache was completely relieved and the visual field deficit improved remarkably after the operation. The authors recommend surgical management for pediatric RCC apoplexy patients who present with severe neuro-ophthalmic signs or deterioration of consciousness, although there are at present no standardized management guidelines for pediatric RCC apoplexy.

Symptomatic infratentorial ependymal cyst arising from the medulla: a case report with review of literature.

BACKGROUND: Ependymal cysts (EC) typically present supra-tentorially near the lateral ventricle, juxta ventricular, or temporoparietal regions. Previous cases have also identified infratentorial EC of the brainstem, cerebellum, and subarachnoid spaces. They are mostly asymptomatic. In this paper, we present the first-ever case of a symptomatic medullary ependymal cyst treated with surgery, along with a comprehensive review of the literature on EC of other parts of the brain stem. CASE DESCRIPTION: This 51-year-old female presented with hearing loss, dizziness, diplopia, and ataxia. Radiographic imaging indicated the presence of a non-enhancing lesion in the medulla with a mass effect on the brainstem. Pathological examination confirmed its characterization as an ependymal cyst. The patient underwent a suboccipital craniotomy for the fenestration of the medullary ependymal cyst under neuro-navigation, Intra-op ultrasound and intra-operative neuro-monitoring. Histopathological examination confirmed the diagnosis of an ependymal cyst. At one month follow-up, her KPS is 90, ECOG PS 1, and her ataxia has improved with complete resolution of diplopia. CONCLUSION: Due to their rarity and potential similarity to other cystic structures, EC may be overlooked or incorrectly diagnosed resulting in mismanagement and surgical disaster. Therefore, a comprehensive understanding and awareness of their distinct characteristics are essential for accurate diagnosis and appropriate management.

The 1 µg Synacthen stimulation test in the diagnosis of secondary adrenal insufficiency in patients with Rathke's cleft cyst and empty sella syndrome.

INTRODUCTION: Rathke's cleft cyst (RCC) and primary empty sella syndrome (PESS) are usually incidental findings on magnetic resonance imaging (MRI) scans. In most cases, these lesions do not cause mass effect symptoms and do not require surgical intervention. In patients with RCC or PESS, it is important to exclude secondary adrenal insufficiency (SAI), which may be a life-threatening condition. MATERIAL AND METHODS: The incidence of SAI was assessed in patients with RCC or PESS detected by MRI, using the 1 µg Synacthen stimulation test. A total of 38 patients were analysed. Test results were linked to clinical symptoms and the type of cystic lesion. RESULTS: Assuming that cortisol levels < 14.6 µg/dL in Synacthen test are the criterion of SAI diagnosis, SAI was diagnosed only in 2 patients (5%). Adopting the traditional criterion of cortisol levels < 18 µg/dL, SAI would be diagnosed in 7 patients (18.4 %). Dizziness (Chi2 = 3.89; p = 0.049) and apathy (Chi2 = 3.87; p = 0.049) were significantly more frequent in the PESS group than in the RCC group. CONCLUSIONS: The incidence of SAI in the general patient population with empty sella syndrome and Rathke's cleft cysts is low. The 1 µg Synacthen test seems to be a valuable tool in the diagnosis of SAI among patients with RCC and PESS. Further studies are necessary to determine the sensitivity and specificity of the 1 µg Synacthen test with the standardization of test protocol and considering the cortisol level at the 20-minute timepoint. PESS patients report dizziness and apathy more frequently than RCC patients, which does not result from the disturbance of the hypothalamic-pituitary-adrenal axis, but probably from the different pathogenesis of these cystic lesions.

Quiste de bolsa de Rathke e hiperprolactinemia, una causa infrecuente de amenorrea primaria / Rathke cleft cysts and hyperprolactinemia, an uncommon cause of primary amenorrhea

El quiste de la bolsa de Rathke es una lesión epitelial benigna de la región selar, formada a partir de remanentes embrionarios. La mayoría de los casos son asintomáticos, aunque pudiera presentarse con cefalea, disfunción hipofisaria y trastornos visuales, muy infrecuentemente como apoplejía hipofisaria. Se presenta el caso de una paciente que, habiendo presentado amenorrea primaria, se le realiza el diagnóstico de quiste de la bolsa de Rathke con hiperprolactinemia, logrando menarquia luego del tratamiento con cabergolina.

Rathke's cyst is a benign epithelial lesion of the sellar region, formed from embryonic remnants. Most cases are asymptomatic although it could present with headache, pituitary dysfunction and visual disorders, very infrequently as pituitary stroke. We present the case of a patient who, having presented primary amenorrhea, is diagnosed with Rathke's cyst with hyperprolactinemia, achieving menarche after treatment with cabergoline.

Neurocisticercosis o hidatidosis: difícil diagnóstico diferencial ante un quiste parasitario cerebral / Wether neurocysticercosis or hydatid disease: difficult differential diagnosis towards a parasitic brain cyst

Los quistes parasitarios cerebrales son una patología frecuente en áreas endémicas, pero en los últimos años está en aumento en otras regiones por efecto de la inmigración. Dos de las enfermedades que de forma más habitual producen parasitosis cerebral son la hidatidosis y la neurocisticercosis. El cuadro clínico en ambas entidades es similar, por lo que son de utilidad las pruebas de imagen y la serología para hacer el diagnóstico diferencial. Describimos el curso clínico de un niño de 9 años de edad con síntomas de proceso expansivo cerebral y prueba de imagen compatible con quiste parasitario, en el que se inicia tratamiento antiparasitario y, tras varios meses de evolución, se consigue la remisión paulatina de la sintomatología y el tamaño del quiste (AU)

Parasitic brain cyst is a frequent pathology in endemic areas, but in recent years this disease is increasing in other regions as a result of immigration. Two of the most common diseases that cause brain parasites are hydatidosis and neurocysticercosis. The clinical picture is similar in both entities, for that reason, imaging tests and serology are useful for differential diagnosis. We describe the clinical course of a 9-year-old boy with symptoms of cerebral expansive process. The imaging tests are compatible with parasitic cyst. We initiate antiparasitic treatment and after several months of development, we got the gradual remission of symptoms and the size of the cyst was decreasing (AU)

Hallazgo por neuroimaginología de microangiopatía cerebral retiniana con calcificaciones y quistes / Neuroimaging findings in cerebroretinal microangiopathy with calcifications and cysts

La microangiopatía cerebral retiniana con calcificaciones y quistes es una enfermedad poco frecuente, caracterizada por alteraciones cerebrales, retinianas y óseas, así como por predisposición al sangrado gastrointestinal. Existen pocos reportes de casos de esta condición, especialmente en adultos, en quienes la incidencia es baja. Los hallazgos por medio de neuroimágenes son característicos, con calcificaciones bilaterales y múltiples formaciones quísticas. El propósito de este artículo fue hacer una revisión bibliográfica e ilustrar dos casos cuyo diagnóstico fue posible con la ayuda de neuroimágenes.

Cerebroretinal microangiopathy with calcifications and cysts is a rare condition characterized by brain, retinal and bone anomalies, as well as a predisposition to gastrointestinal bleeding. There are few reported cases of this condition in adults, among whom the incidence is low. Neuroimaging findings are characteristic, with bilateral calcifications, leukoencephalopathy and intracranial cysts. The purpose of this article was to do a literature survey and illustrate two cases diagnosed with the aid of neuroimaging.

Cisto epidermoide da fossa posterior: relato de caso / Epidermoid cyst of the posterior fossa: a case report

Epidermoid cysts of the central nervous system are uncommon conditions, which are frequently located in the cerebellopontine angle and around the pons. They are covered with keratinized squamous epithelium and keratin lamella, which give its contents a soft, white-pearly appearance. Epidermoid cysts are mostly originated from malformations, presumably associated with surface elements of the nervous system ectoderm during the closure of the neural groove or formation of secondary cerebral vesicles. The authors describe a case of epidermoid cyst in the posterior fossa causing hydrocephalus and review morphologic and diagnostic criteria of this lesion.

Os cistos epidermoides do sistema nervoso central (SNC) são condições incomuns, estando localizados mais frequentemente no ângulo pontocerebelar e ao redor da ponte. Eles são revestidos por epitélio escamoso queratinizado e lamelas de queratina, tornando seu conteúdo branco-perolado e pastoso. Os cistos epidermoides são, na maioria das vezes, originados de malformações, possivelmente associados ao entremeio de elementos superficiais do ectoderma do SNC durante o fechamento da placa neural, ou formação das vesículas cerebrais secundárias. Os autores descrevem um caso de cisto epidermoide da fossa posterior determinando hidrocefalia e revisam critérios morfológicos e diagnósticos dessa lesão.

Quiste glioependimario congenito: reporte de caso / Glioependymal congenital cyst: case report

El Quiste Glioependimario es un tumor extremadamente raro y excepcionalmente pocos casos han sido reportados; se caracterizan por una cavidad cerrada, con una capa de tejido de epitelio ependimario o una mezcla de tejido ependimario y epitelio coloideo, con un lumen relleno por líquido cefalorraquídeo. Presentamos el caso de un paciente manejado en el servicio de neurocirugía de la Universidad de Cartagena con esta patología.

Rathke's cleft cyst and partial feet adactyly: an unusual association

A 53 year-old woman presented a recurrent bifrontal headache of 2 years duration and bilateral progressive visual disturbance. The clinical and neurological examination showed a bilateral feet adactyly and bitemporal hemianopsia. The brain MRI demonstrated a Rathke's cleft cyst. The patient was operated by a transnasal endoscopic approach. It seems that this unusual association has never been described before.

Mulher de 53 anos com história recorrente de cefaléia com duração de 2 anos bilateral e progressiva, acompanhada de distúrbios visuais. O exame clinico e neurológico mostrou uma adactilia dos pés e hemianopsia bitemporal. A ressonância nuclear magnética cerebral mostrou um cisto de Rathke. A paciente foi operada por via transnasal endoscópica. Aparentemente esta é a primeira vez que esta associação é descrita na literatura.

Cistos de bolsa de Rathke: diagnóstico e tratamento / Rathke's pouch cysts: diagnosis and treatment

Estudamos retrospectivamente quatro pacientes com cisto de bolsa de Rathke. Chamamos a atençäo para a apresentaçäo clínica e para o tratamento destas lesões. Todos ocorreram em pacientes do sexo feminino. Os sintomas predominantes foram amenorréia, cefaléia e déficit visual. Revisamos várias séries publicadas de cistos de bolsa de Rathke. Concluimos que o tratamento cirúrgico é ainda a melhor conduta e que a via transesfenoidal é a abordagem mais indicada

Síndrome de Diógenes: hallazgos neurorradiológicos en sustancia blanca. Base orgánica de las alteraciones del autocuidado en personas de edad avanzada / Diogenes syndrome: neuroradiology findings in white matter. Organic basis of self-care alterations in elderly people

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