Anomalias venosas nos cavernomas / Venous anomalies in cavernomas

As anomalias do desenvolvimento venoso (DVA) são as malformações vasculares cerebrais mais frequentes, tendo geralmente um curso benigno, e podem estar acompanhando os cavernomas. Atualmente, são mais diagnosticadas por causa dos métodos de diagnósticos por imagem que existem, como a tomografia computadorizada e, sobretudo, a ressonância magnética nuclear. Este artigo revisa as DAV em geral e as relacionadas com os cavernomas. Os artigos foram buscados na base de dados PubMed, em publicações desde 1995 a junho de 2012, usando como filtros as expressões ?anomalias do desenvolvimento venoso e malformações cavernosas? e ?anomalias venosas cerebrais e malformações cavernosas?; a pesquisa foi limitada a artigos em idioma inglês e relacionados a humanos. As sessões de referência de artigos e revisões recentes foram revisadas e os artigos pertinentes foram recuperados no formato resumo; os manuscritos em texto completo foram obtidos subsequentemente para todos os artigos originais aplicados à revisão em curso...

Developmental venous anomalies (DAV) are the most common brain vascular malformations usually, having a benign course and may be following the cavernomas. They are more diagnosed recently due to diagnostic imaging methods that exist, such as computed tomography and nuclear magnetic resonance. This article reviews the DAV in general and those relating to cavernomas. The PubMed database was searched for publications from 1995 through June 2012 using the mesh terms ?anomalies venous cerebral, development venous and cavernous malformation?, ?anomaly venous cerebral and cavernous malformation?. The search was limited to articles in the English language and related to humans subjects. Reference sections of recent articles and reviews were reviewed and pertinent articles were retrieved in the abstract format, full text manuscripts were subsequently obtained for all original articles applicable to the current review...

Congenital Pial Arteriovenous Fistula in the Temporal Region Draining into Cavernous Sinus: A Case Report

This report concerns a 4-month-old infant with progressive prominent and redness of his left eye since birth. This report concerns a 4-month-old infant with progressive prominent redness of his left eye since birth. Angiography revealed a congenital pial arteriovenous fistula between the temporal branch of the left posterior cerebral artery and left cavernous sinus through the sphenoparietal sinus, a condition not reported in the literature. The fistula was successfully occluded with two micro-coils by vertebrobasilar approach.

Perioperative care in brain arteriovenous malformations / Cuidados perioperatórios em malformações arteriovenosas encefálicas

The authors provide a review of brain arteriovenous malformations, initially reviewing epidemiological and etiological aspects in addition to the pathophysiology and risk factors associated with bleeding. The emphasis of this review is directed to the clinical and care should be taken since the diagnosis of this pathology, intraoperative management on the viewpoint of the anesthesiologist to the potential complications that occur after resection of the lesion.

Os autores realizam uma revisão sobre malformações arteriovenosas encefálicas, revisando inicialmente aspectos etiológicos e epidemiológicos, além da fisiopatologia e de fatores relacionados com risco de sangramento. A maior ênfase dessa revisão é direcionada para o quadro clínico e para os cuidados que devem ser tomados desde o diagnóstico dessa patologia, o manejo intraoperatório sob o ponto de vista do anestesista, até as possíveis complicações que ocorrem após a ressecção da lesão.

Autodestrucción espontánea de una malformación arteriovenosa cerebral / Spontaneous self-destruction of a cerebral arteriovenous malformation

Se presenta una paciente que fue investigada y operada en el Hospital Universitario Gral Calixto García, portadora de una malformación arteriovenosa cerebral, que desapareció espontáneamente después de un cuadro neurológico de intensa gravedad. Arteriográficamente se comprobó que estaba asociada a un aneurisma arterial en su mismo sistema vascular cerebral. La paciente había sufrido 3 eventos neurológicos en orden ascendente de gravedad, y fue el último motivo de ingreso en la Unidad de Terapia Intensiva, con un nivel de 5 en la escala de Glasgow para el coma y sin signos meníngeos evidenciables. La lesión valorada como malformación arteriovenosa cerebral en las tomografías axiales cumputarizadas previas, no se evidenció en la panangiografía cerebral realizada en la fase de mejoría clínica relativa después de su etapa más crítica. Dicha angiografía demostró un aneurisma arterial homolateral a la malformación arteriovenosa ya desaparecida, el cual fue excluido de la circulación por presillamiento de su cuello sin eventos transoperatorios ni posoperatorios relevantes. Se realiza una revisión de la literatura al respecto(AU)

Authors present the case of a patient researched and operated on in the General Calixto García University Hospital presenting with a cerebral arteriovenous malformation disappeared spontaneously after a neurologic picture of intense severity. From the arteriographic point of view it was verified that it was associated with an arterial aneurysm present in her same cerebral vascular system. The patient has had three neurological events in rising order of severity which was the last reason for admission in the Intensive Therapy Unit (ITU) with a level of 5 in the Glasgow scale for the coma and without obvious meningeal signs. The injury classified as a cerebral arteriovenous malformation according to the previous computerized axial tomographies was not evidenced in the cerebral panangiograpy carried out in the clinical phase improvement after its more critical stage. Such angiography demonstrated the presence of a arterial aneurysm homolateral to the already disappeared arteriovenous malformation, which was excluded of the circulation by means of stitching of neck without transoperative and postoperative significant events. In this respect, author made a literature review(AU)

Brainstem cavernous malformations: a review with two case reports / Malformações cavernosas do tronco cerebral: uma revisão com relato de dois casos

Central nervous system (CNS) cavernous malformations (CMs) are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15 percent of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.

Malformações cavernosas (MFC) do sistema nervoso central são malformações do desenvolvimento do leito vascular com múltiplas apresentações clínicas devido a sua natureza dinâmica. Apresentamos dois casos de malformações cavernosas do tronco cerebral: o primeiro após radioterapia e o segundo em paciente grávida com hemorragia intracraniana sintomática. MFC são responsáveis por cerca de 8-15 por cento de todas as malformações vasculares. Embora tradicionalmente sejam genéticas, as MFC podem também ser adquiridas, particularmente após radioterapia. As manifestações clínicas são variáveis e o tratamento cirúrgico deve ser considerado para pacientes com quadros neurológicos progressivos.

Hemangioma racemoso de retina (síndrome de Wyburn-Mason): acompanhamento de um paciente por dez anos: relato de caso / Retinal racemose hemangioma (Wyburn-Mason syndrome): a patient ten years follow-up: case report

Relatar o caso de um paciente que apresentou hemangioma racemoso de retina (síndrome de Wyburn-Mason) e seu acompanhamento por 10 anos. Dez anos após o diagnóstico da doença o exame oftalmológico não sofreu alterações, assim como o campo visual e a retinografia. O prognóstico a longo prazo é controverso. Alguns autores relatam estabilidade das lesões oculares, como no caso descrito, enquanto outros referem perda progressiva da visão.

To describe the case of a patient who presents retinal racemose hemangioma (Wyburn-Mason syndrome) and his 10 years follow-up. Ten years after the disease diagnosis, the ophthalmologic exam had no changes, as well as the campimetry and the retinography. The long term prognosis is controversial. Some authors report ocular lesions stability and others report progressive visual loss.

Las malformaciones arteriovenosas cerebrales / The cerebral arteriovenous malformations

Las malformaciones arteriovenosas cerebrales se han encontrado en 0.6% de las autopsias y su mortalidad es alta. Clínicamente se presentan como hemorragias intracraneales en el 50% de los casos. Otros síntomas son cefalea, crisis convulsivas y déficits neurológicos agudos. La mayoría de los pacientes son asintomáticos al nacimiento e inician síntomas entre la segunda y la cuarta década de la vida. El diagnóstico se basa en estudios de neuroimagen y arteriografía. El manejo de las MAV del cerebro debe ser multidisciplinario. El tratamiento actual incluye tres opciones con orden ascendente de invasividad: radiocirugía, terapia endovascular (embolización) y resección microquirúrgica. La decisión del tipo de tratamiento deberá individualizarse y depende de la evolución de la lesión y el riesgo relacionado con cada modalidad de tratamiento...

Detection of Residual Brain Arteriovenous Malformations after Radiosurgery: Diagnostic Accuracy of Contrast-Enhanced Three-Dimensional Time of Flight MR Angiography at 3.0 Tesla

OBJECTIVE: Although three-dimensional time-of-flight magnetic resonance angiography (3D TOF-MRA) is used frequently as a follow-up tool to assess the response of arteriovenous malformations (AVMs) after radiosurgery, the diagnostic accuracy of 3D TOF-MRA is not well known. We evaluated the diagnostic accuracy of contrast-enhanced 3D TOF-MRA at 3.0 Tesla for the detection of residual AVMs. MATERIALS AND METHODS:This study included 32 AVMs from 32 patients who had been treated with radiosurgery (males/females: 21/11; average patient age, 33.1 years). The time interval between radiosurgery and MRA was an average of 35.3 months (range, 12-88 months). Three-dimensional TOF-MRA was obtained at a magnetic field strength of 3.0 Tesla after infusion of contrast media, with a measured voxel size of 0.40 x 0.80 x 1.4 (0.45) mm3 and a reconstructed voxel size of 0.27 x 0.27 x 0.70 (0.05) mm3 after zero-filling. X-ray angiography was performed as the reference of standard within six months after MRA (an average of two months). To determine the presence of a residual AVM, the source images of 3D TOF-MRA were independently reviewed, focusing on the presence of abnormally hyperintense fine tangled or tubular structures with continuity as seen on consecutive slices by two observers blinded to the X-ray angiography results. RESULTS: A residual AVM was identified in 10 patients (10 of 32, 31%) on X-ray angiography. The inter-observer agreement for MRA was excellent (kappa= 0.813). For the detection of a residual AVM after radiosurgery as determined by observer 1 and observer 2, the source images of MRA had an overall sensitivity of 100%/90% (10 of 10, 9 of 10), specificity of 68%/68% (15 of 22, 15 of 22), positive predictive value of 59%/56% (10 of 17, 9 of 16), negative predictive value of 100%/94% (15 of 15, 15 of 16) and diagnostic accuracy of 78%/75% (25 of 32, 24 of 32), respectively. CONCLUSION: The sensitivity of contrast-enhanced 3D TOF-MRA at 3.0 Tesla is high but the specificity is not sufficient for the detection of a residual AVM after radiosurgery.

Malformações arteriovenosas encefálicas: aspectos morfológicos, clínicos e operatórios. Revisão da literatura / Intracranial arteriovenous malformation. A literature review

É feita revisão da literatura sobre malformações arteriovenosas(MAV)encefálicas. MAV são anomalias morfológicas neurovasculares caracterizadas por comunicação direta entre artérias e veias , sem interposição do leito capilar, portanto sem resistência ao fluxo sanguíneo. Morfologicamente, MAV possuem três componentes distintos:aferências, nidus e eferências.As aferências incluem vasos originados de quaisquer artérias intra ou extracranianas que nutrem as MAV.O nidus corresponde a um enovelado complexo de artérias e veias anormais,unidas por uma ou mais fístulas além de calcificações e aneurismas intranidais. As eferências constituem as drenagens venosas, que podem ser superficial,profunda ou mista.Acidentes vasculares encefálicos(AVE)hemorrágicos são as manifestações mais comuns,ocorrendo em aproximadamente 50 por cento dos casos.O tratamento de MAV pode ser microcirurgia,embolização endovascular,radiocirúrgico estereotáxico,conservador ou multidisciplinar.Ressecção cirúrgica completa de MAV é considerada a melhor opção de tratamento,eliminando risco de futura hemorragia.Apesar do desenvolvimento tecnológico na área de neurocirurgia endovascular nas últimas décadas, a fisiopatologia de MAV e sua história natural ainda não estão bem esclarecidas.O tratamento representa um grande desafio.Acreditamos que estudos sobre complexidade arquitetônica e hemodinâmica de MAV são necessários a fim de estabelecer fatores de risco e prognósticos,melhorando os resultados do tratamento.

Malformación de la vena de Galeno / Vein of Galen malformation

Clasificación de esta lesión, que consiste en la malformación vascular cerebral que con mayor frecuencia se diagnostica en el recién nacido. También se describen sus signos clínicos, y aspectos de su diagnóstico ecográfico.

The Rare Association of Moyamoya Disease and Cerebral Arteriovenous Malformations: a Case Report

A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow-up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.

Transarterial Embolization with Berenstein Liquid Coils and N-butyl Cyanoacrylate in a Vein of Galen Aneurysmal Malformation: a Case Report

A 12-week-old baby with a vein of Galen aneurysmal malformation (VGAM) was successfully treated with performing transarterial microcatheter-directed embolization with Berenstein Liquid Coils and n-butyl cyanoacrylate in the feeding arteries. Post-procedure angiography showed a marked decrease of the blood flow into the dilated vein of Galen. Three months later, follow-up angiography showed that the vein of Galen aneurysmal malformation had totally disappeared, and the baby recovered very well without any sequelae. We report here on this interesting case along with a review of the relevant literature, and we aim to enhance physicians' awareness of the treatment for VGAMs.

Vein of Galen aneurysm in an adult: case report / Aneurisma da veia de Galeno em adulto: relato de caso

Vein of Galen aneurysm is a rare pathology, representing less than 1 percent of intracranial vascular malformations. We report on a 65 years-old man who experienced a generalized tonic-clonic seizure. Brain imaging showed a large calcified expanding mass in the pineal region, confirming the diagnosis of a vein of Galen aneurysm. Because of the spontaneous thrombosis of the malformation, there was no need for microsurgical or endovascular treatment and he is been regularly followed since that.

Aneurisma da veia de Galeno é patologia rara, representando menos de 1 por cento das malformações vasculares intracranianas. Apresentamos o caso de um homem de 65 anos que teve episódio de crise convulsiva tônico-clônica generalizada. Exames de imagem evidenciaram grande processo expansivo calcificado na região pineal, confirmando o diagnóstico de aneurisma trombosado de veia de Galeno. Devido à trombose espontânea da malformação, foi excluída a possibilidade de tratamento endovascular bem como microcirúrgico, mantendo-se o acompanhamento clínico.

Malformación arteriovenosa cerebelosa / Cerebellar arteriovenous malformation

Se presenta el caso de una paciente de sexo femenino de 20 meses de edad que consultó al servicio de urgencias pediátricas por cuadro de pérdida súbita y progresiva de la fuerza del hemicuerpo izquierdo, asociada con episodio convulsivo, pérdida del conocimiento de cerca de 15 segundos, astenia, adinamia, irritabilidad y somnolencia. Niega trauma craneoencefálico. La presentación de este caso y su estudio posterior obliga a la revisión de las causas más frecuentes de cuadros convulsivos en niños, síndromes hemipléjicos y hemiparéticos, con el fin de enfocar un buen diagnóstico y diseñar un plan de manejo adecuado enfocado hacia las malformaciones vasculares cerebrales o cerebelosas.

Utilidad del Doppler color en el diagnóstico de una fístula arterio-venosa dural: presentación de un caso clínico en período neonatal / Utility of the color Doppler in the diagnosis of a dural arteriovenous fistula: presentation of a clinical case in neonatal period

Presentamos el caso clínico de un recién nacido de 35 semanas de gestación con sospecha prenatal de un tumor cerebral. El cuadro clínico del recién nacido orientó hacia una malformación arteriovenosa intracraneal lo que se demostró mediante ultrasonografía con Doppler color efectuada en la UTI neonatal. Se destaca la buena correlación diagnóstica con la arteriografía cerebral efectuada por cateterismo femoral que precisó la existencia de un fístula dural gigante, presentando los buenos resultados del tratamiento mediante embolización con microcoils, que permitió la sobrevida del niño con mínima secuela, a los dos años de seguimiento.