Quiste aracnoideo sacro recidivante con meningocele anterior gigante: reporte de caso / Sacral arachnoid recurrent cyst with giant anterior meningocele: a case report

Los quistes aracnoideos corresponden a lesiones benignas expansivas del canal medular secundarias a defectos anatómicos durales, mientras que los meningoceles anteriores consisten en la herniación de la duramadre hacia la pelvis a través de forámenes dilatados o defectos óseos. Ambas entidades son infrecuentes y sus manifestaciones clínicas puede variar de acuerdo a estructuras anatómicas que comprimen. Una historia clínica completa, la pesquisa diagnóstica y la adecuada interpretación de imágenes orientan al diagnóstico y manejo de estos pacientes. Se presenta el caso de una paciente adulta mayor con historia de masa pélvica, dolor lumbar severo y monoparesia en quien se realizó el diagnóstico incidental de quiste aracnoideo sacro y meningioma sacro anterior.

Arachnoid cysts are benign expansive lesions of the spinal canal secondary to dural defects, whereas the anterior meningoceles consist of the herniation of the dura into the pelvis through dilated foramina or bone defects. Both pathologies are infrequent and its clinical manifestations vary according to compressed anatomical structures. A complete clinical history, the diagnostic investigation and the correct imaging studies interpretation guide the diagnosis and management of these patients. We present the case of an elderly adult patient with a history of pelvic mass, severe lumbar pain and monoparesis in whom the incidental diagnosis of sacral arachnoid cyst and anterior sacral meningioma was performed.

Do you know this syndrome? Schimmelpenning-Feuerstein-Mims syndrome

Abstract Nevus sebaceous is the most common type of organoid epidermal nevus, often located on the face, following the Blaschko's lines and with alterations in the ipsilateral central nervous system. Distinct disorders can be distinguished by the type of association with epidermal nevus. Schimmelpenning-Feuerstein-Mims syndrome is a rare multisystem disorder characterized by sebaceous nevus associated with extracutaneous abnormalities affecting the brain, eyes and bones. We report the case of an 8-year-old female patient with a yellowish verrucous plaque on the left temporal area extending ipsilaterally to the cervical region, combined with cicatricial alopecia, periocular nodule, and epibulbar tumors.

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.

Puberdade precoce central como única manifestação de cisto aracnoide supraselar / Central precocious puberty as a sole manifestation of suprasellar arachnoid cyst

OBJETIVO: Os cistos aracnoides são achados raros. Em 10 por cento dos casos, sua localização é supraselar e ocorrem quase exclusivamente em crianças. Em geral, apresentam manifestações neurológicas e visuais, sendo incomum a puberdade precoce central como sinal clínico de sua presença. DESCRIÇÃO DO CASO: Menina avaliada aos dois anos e sete meses por telarca iniciada com um ano e oito meses e pubarca e axilarca com dois anos, com aumento da velocidade de crescimento (13cm/ano) e da idade óssea (sete anos e um mês). Ao exame, apresentou peso de 22,6kg (Z+4,0), estatura de 106cm (Z+3,5) e Tanner de M3P2. Exames realizados: teste de estímulo com GnRH (LH basal 8,3 UI/L, pico aos 30 minutos 94,3 UI/L; FSH basal=10,1 UI/L, pico aos 30 minutos 29,5 UI/L), ressonância nuclear magnética de crânio com cisto aracnoide supraselar e demais testes de função hipofisária normais. Foi iniciado tratamento com análogo de GnRH. Atualmente, aos quatro anos e três meses com estadio puberal M3P2, velocidade de crescimento e desenvolvimento neuropsicomotor normais para a idade. COMENTÁRIOS: A puberdade precoce central pode ser manifestação única de um cisto aracnoide, sendo essenciais o diagnóstico e o tratamento precoces, além de seguimento prolongado, uma vez que outras disfunções hipofisárias podem ocorrer tardiamente.

OBJECTIVE: Arachnoid cysts (AC) are a rare finding; 10 percent of them are suprasellar and occur almost exclusively in children. They are frequently associated with neurological and visual manifestations. Central precocious puberty as a sole manifestation is uncommon. CASE DESCRIPTION: Girl evaluated at the age of two years and seven months. She started breast development at the age of one year and eight months, pubic and axillary hair at the age of two years, with growth velocity acceleration (13cm/year) and increased bone age (seven years and one month). On exam: weight: 22.6kg (Z+4.0), height: 106cm (Z+3.5) and puberal stage of B3P2. The GnRH stimulus test was performed (basal LH 8.3 IU/L, at 30 minutes 94.3 IU/L; FSH basal=10.1 IU/L, at 30 minutes 29.5 IU/L). Nuclear Magnetic Resonance of the skull showed a suprasellar arachnoid cyst. Others stimulation tests were performed and excluded pituitary deficiencies. Treatment with a GnRH analog was started. At the age of four years and three months, she was B3P2, with normal growth velocity and neurologic development. COMMENTS: Central precocious puberty can be the only manifestation of AC. It is essential to establish early diagnosis and treatment. A prolonged follow up is recommended, since late pituitary dysfunctions can occur.

Spontaneous intracystic hemorrhage of the arachnoid cyst: a case report and review of literature.

Arachnoid cysts represent benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane and do not communicate with the ventricular system. We report a case of a years right handed lady, who presented to the emergency department with the complaints of headache and vomiting for one week CT scan showed extraaxial cystic lesion in the left fronto-parietal region. On the fifth day of admission, patient had sudden onset of severe headache associated with loss of consciousness for about 3-4 minutes with neck rigidity. A CT scan of head was repeated, which showed left fronto-parietal cystic lesion with intracystic bleed and SAH. Intraoperatively, there was intradural cystic lesion containing xanthochromic fluid with normal brain surface and there were no evidence of any vascular malformations. Marsupilization of the cystic lesion was carried out and she improved. The literature regarding arachnoid cyst with spontaneous intracranial haemorrhage is reviewed.

Hamartoma hipotalámico asociado a dos quistes aracnoideos: a propósito de un caso / Hypothalamic hamartoma associated with arachnoid cyst: report of a case

Hypothalamic hamartomas are heterotopias of the nervous tissue which are similar to the grey matter of the hypothalamus. They may cause precocious puberty and/or gelastic seizures. We present the case of a patient who began her pubertal development at the age of 9 months. Cranial magnetic resonance imaging showed a hypothalamic hamartoma associated with middle cranial fossa and pineal region arachnoid cysts, a very rare association.

Los hamartomas hipotalámicos son heterotopias de tejido nervioso que se asemejan a la sustancia gris del hipotálamo. Pueden causar pubertad precoz y/o crisis gelásticas. Presentamos el caso de una paciente que a los 9 meses de edad comenzó desarrollo puberal y que en la resonancia magnética craneal se objetivó un hamartoma hipotalámico asociado a dos quistes aracnoideos, uno en fosa craneal media y otro en región pineal, asociación que es muy infrecuente.

Spontaneous acute subdural hematoma contralateral to an arachnoid cyst

Arachnoid cysts (AC) are extra-cerebral cerebrospinal fluid collections of unknown origin. They correspond to 1 percent of all intracranial nontraumatic space-occupying lesions and appear more frequently in the middle fossa (50 percent). More than 25 percent of these cysts are incidental findings and the majority of patients are asymptomatic. Seizures, intracranial hypertension signs, neurological deficits, macrocrania, developmental delay and bulging of the skull are the main signs and symptoms of the lesion. AC rupture and bleeding are rare, usually occurring in young adults and associated with trauma. The risk of hemorrhage does not exceed 0.04 percent / year. We describe the case of a ten-year-old boy who presented with acute signs of intracranial hypertension secondary to a spontaneous acute subdural hematoma, contralateral to an AC of the middle fossa. Three factors were significant in this case: signs and symptoms occurred spontaneously; the presence of an acute subdural hematoma exclusively contralateral to the AC; successful outcome of the conservative treatment.

Os cistos aracnóideos (CA) são coleções liquóricas extra-cerebrais e intra-aracnóideas de origem desconhecida. Correspondem a 1 por cento de todas as lesões expansivas intracranianas não traumáticas e têm nítido predomínio na fossa média (50 por cento). Até 25 por cento destes cistos são achados incidentais sendo que a maioria dos pacientes é assintomática. Crises epilépticas, sinais de hipertensão intracraniana, déficits neurológicos focais, macrocrania, atraso no desenvolvimento e abaulamento da calota craniana são os principais sinais e sintomas da lesão. A ruptura dos CA, assim como seu sangramento, são situações raras, geralmente associadas a traumas e acometem adultos jovens. O risco de hemorragia em pacientes com CA não excede 0,04 por cento ao ano. É descrito caso de paciente de dez anos de idade que subitamente apresentou sinais de hipertensão intracraniana secundários a hematoma subdural agudo espontâneo, contralateral a volumoso CA de fossa média. Três fatores foram de relevância neste paciente: a ocorrência de sinais e sintomas espontaneamente, especialmente em um menino; presença de hematoma subdural agudo exclusivamente contralateral ao CA; e a boa evolução com o tratamento conservador.

Cisto aracnóide intracraniano: revisão de literatura / Arachnoid cysts: a review of the literature

Objetivo: Revisão da literatura sobre os cistos aracnóides intracranianos – formação e evolução, dados epidemiológicos e clínicos, aspectos de neuroimagem e tratamento. Método: Realizamos pesquisa bibliográfica utilizando como base de dados o MEDLINE (National Library of Medicine), abrangendo operíodo de 1980 a 2006. As palavras-chave utilizadas foram arachnoid cysts e intracranial cysts. Também realizamos pesquisa manual em jornais e revistas médicas. Artigos com dados clínicos incompletos não foram incluídos no trabalho. Conclusões: Embora haja um consenso sobre a intervenção cirúrgica para os casos sintomáticos, a abordagem ideal ainda é controversa.

Objective: To review the current literature on intracranial arachnoid cysts related to its formation,evolution, the epidemiological, clinical and neuro imaging aspects and also its treatment. Method:Bibliographic review was done using Medline data base from 1980 to 2006. The key words used were arachnoid cysts and intracranial cysts. Manual research on pertinent medical publications was also done.Articles with incomplete clinical data were not included. Conclusions: Although there is a consensus that surgery should be performed only in the symptomatic cases, there is no general agreement whichis the best surgical approach to deal with arachnoid cysts.

Multiple cavernous malformations with supravermian arachnoid cyst.

Cerebral cavernous malformation are congenital vascular abnormalities that have been reported in 0.4% of the population; they represent 5-13% of all cerebrovascular malformations. Onset of cerebral cavernous malformations may be associated with seizures, intracranial hemorrhages, focal neurological deficit or migraine-type headaches. Some patients may require surgical intervention due to hemorrhage. Multiple cavernomas in childhood have been reported in the literature, but they are rare. This manuscript presents a 12-year-old girl with multiple cavernomas accompanied by supravermian arachnoid cyst detected by neuroimaging techniques. This is the first report that demonstrates a case of pediatric multiple cavernous malformation coexisting with arachnoid cyst of the supravermian cistern.

Achado ocasional de doenças neurológicas durante a pesquisa da surdez infantil através do BERA / Occasional finding of neurological disorders during children hearing loss evaluation using the ABR

Uma das mais importantes aplicações clínicas dos potenciais evocados auditivos de tronco cerebral (BERA) é a sua utilização na avaliação da surdez infantil. Atualmente o BERA também é utilizado na triagem das síndromes cócleo-vestibulares a procura de lesões retro-cocleares, na monitoração dos estados de coma (morte cerebral), na monitoração do tronco cerebral em cirurgias da base do crânio, etc. Uma das qualidades do BERA é a sua capacidade de avaliar a integridade neurofisiológica das vias auditivas do tronco cerebral. Desta maneira, algumas vezes durante a pesquisa dos limiares auditivos infantis, nos deparamos com BERAs que sugerem a presença de lesões retro-cocleares das vias auditivas (assimetria de traçados, aumento dos intervalos interpicos), muitas vezes comprovadas através de exames de imagem. Trata-se de achado ocasional de moléstia neurológica por ocasião da pesquisa dos limiares auditivos infantis. Neste trabalho relataremos dois casos de doenças neurológicas, diagnosticadas ocasionalmente através do BERA realizado com o intuito de se pesquisar os limiares auditivos.

One of the most important applications of the Brainstem evoked response audiometry (ABR) is in the evaluation of hearing loss in children. Today the ABR is also indicated in the screening of cochleo-vestibular syndromes to detect retrocochlear lesions, to monitor patients in a coma (brain death), in monitoring the brainstem during skull base surgery, etc. Among the many BERA qualities, is its capacity to evaluate the neurophysiologic integrity of the auditory brainstem pathway. In doing so, sometimes while evaluating hearing function in children we are faced with ABR waves that suggest the presence of retrocochlear lesions (trace asymmetry, increased interpeak intervals), many times confirmed through image studies. These cases are seen as occasional findings of neurologic disorders during children hearing loss evaluation. In this study we report 2 cases of neurologic disorders diagnosed with the use of the ABR to evaluate hearing loss in children.

Arachnoid cyst with growth hormone deficiency.

On clinical grounds, arachnoid cysts are usually associated with neurological dysfunction. Little is known concerning their involvement in endocrine disorders. A seven-year-old boy was admitted to the hospital for evaluation of an unprovoked afebrile seizure. His neurological examination was normal, however, he had growth retardation. Insulin tolerance and L-dopa growth hormone stimulation tests revealed an inefficient growth hormone response. An MRI of hypophysis and cranium yielded a shift of hypophysis and a large arachnoid cyst.

Tuberculous radiculomyelitis (arachnoiditis) associated with tuberculous meningitis.

Abstract. A 17-year-old man who presented with progressive quadriparesis is reported. About 8 months prior to admission, he had miliary tuberculosis, and that improved with anti-tuberculous therapy. He had also developed tuberculous meningitis and tuberculous myelitis, respectively. He regularly took anti-tuberculous drugs until this illness. Neurological findings were compatible with cervical cord lesion. CSF analysis indicated a predominate lymphocytic pleocytosis with a high protein level and low sugar profile. MRI findings revealed a multi-loculated arachnoid cyst at C1-C3 level with pressure affecting the adjacent spinal cord and evidence of myelitis at C3-T1 level. Hemi-larminectomy and removal of the arachnoid cyst were performed, but without improvement. A CSF culture yielded M. tuberculosis, that was susceptible to anti-tuberculous drugs.

Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases.

We report a series of three patients with suprasellar arachnoid cysts who presented with a rare 'bobble-head doll' syndrome. The abnormal head movements improved after surgical evacuation of the cysts in all the three cases. Various pathophysiological mechanisms involved in the bobble-head doll syndrome are discussed. The literature on suprasellar arachnoid cysts is briefly reviewed.

Spinal compression caused by multiple arachnoidal cyst.

A 14-year-old boy presented with subacute onset of paraparesis. The spinal compression was due to multiple giant sized spinal arachnoid cysts. Postoperatively the patient recovered completely.

Aneurisma cerebral e cisto de aracnóide: a propósito de caso com hemorragia intracística / Cerebral aneurysm and arachnoid cyst: about a case with intracystic hemorrhage

Relatamos um caso de aneurisma da bifurcação da artéria carótida interna, cuja ruptura se deu para dentro de cisto de aracnóide da fissura silviana. Em revisão da literatura apenas 3 casos foram descritos. Discutimos ainda os aspectos clínicos atípicos do caso, as características dos achados cirúrgicos e uma correlação etiopatogênica entre as duas patologias.

Retrocerebellar arachnoid cyst with syringomyelia: a case report.

Association of syringomyelia with retrocerebellar arachnoid cysts is rare. A case of 14 year old female is being reported, who presented with hydrocephalus caused by a large midline retrocerebellar infravermal arachnoid cyst leading to obstruction of the outlet foramina of the fourth ventricle. There was associated syringomyelia. The pathogenesis of syringomyelia is discussed. The need to evaluate cervical spinal cord by taking T1 weighted sagittal sections in all the patients of large posterior fossa mass lesions causing obstruction to the outlet foramina of the fourth ventricle has been stressed, in order to detect associated syringomyelia.