Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients / Impacto das características angioarquiteturais das malformações arteriovenosas cerebrais na apresentaçáo clínica: análise de 183 pacientes

ABSTRACT Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

RESUMO Antecedentes: A correlação entre a angioarquitetura e a apresentação clínica da Malformação Arteriovenosa do cérebro (MAVc) permanece um assunto de debate. Objetivos: Correlacionar as características angioarquiteturais das MAVc com a apresentação clínica. Métodos: Estudou-se pacientes consecutivos atendidos no Hospital Beneficência Portuguesa-SP, entre 2006 a 2016. Após análise geral, criaram-se cinco grupos de acordo com a apresentação clínica: 1- Hemorragia; 2 - Epilepsia; 3 - Cefaléia; 4 - Déficit Neurológico Progressivo (DNP) e 5 - Incidental. Características epidemiológicas (clínica e topografia) e angioarquiteturais (Classificação de Spetzler-Martin Modificada - SMM; Fluxo intranidal; Aneurismas arteriais, intranidais e venosos; Ectasia venosa; Congestão venosa; "Roubo" arterial; Vascularização dural; Drenagem Venosa Profunda) foram analisadas. Resultados: 183 pacientes foram incluídos e analisados globalmente. Após essa etapa, foram divididos nos grupos: 1 - 56 casos (30,6%); 2 - 49 casos (26,7%); 3 - 41 casos (22,4%); 4 - 28 casos (15,3%) e 5 - 9 casos (4,9%). Principais achados foram referentes a apresentação hemorrágica, na qual observamos correlação estatística positiva com o sexo feminino (P<0,02), lesões classificadas como SMM 3B (P<0,0015) e baixo fluxo (P<0,04). Relacionado à epilepsia, encontramos significância estatística que possibilitou a correlação com pacientes com idade inferior a 36 anos (P<0,001), sexo masculino (P<0,018), lesões superficiais (P<0,002), presença de ectasia venosa (P<0,003) e "roubo" arterial (P<0,01). Pacientes com DNP se apresentam com idade superior aos demais (P<0,01). Conclusões: Após análise multivariada, foi possível separar as MAV em grupos de acordo com as características angioarquiteturais, comprovando que algumas dessas características estão fortemente relacionadas a determinada manifestação.

Abordagem anestésica de grávida com malformação arteriovenosa cerebral e hemorragia subaracnoidea durante a gravidez: relato de caso / Anesthetic approach of pregnant woman with cerebral arteriovenous malformation and subarachnoid hemorrhage during pregnancy: case report / Abordaje anestésico de embarazada con malformación arteriovenosa cerebral y hemorragia subaracnoidea durante el embarazo: relato de caso

JUSTIFICATIVA E OBJETIVOS: A ocorrência de hemorragia subaracnoidea (HSA) durante a gravidez é rara, sendo que cerca de metade é devida a malformações arteriovenosas (MAV). Os autores descrevem a abordagem anestésica de uma grávida de 39 semanas proposta para cesárea, com história de HSA por MAV às 22 semanas. RELATO DO CASO: Grávida de 39 semanas, saudável previamente à gravidez, com antecedentes de HSA às 22 semanas de gestação, manifestada por cefaleias, vômitos e tonturas, sem perda de consciência ou outros déficits à admissão no serviço de urgência. A ressonância magnética (RM) revelou MAV frontal esquerda. Após curto internamento para estabilização e diagnóstico, decidiu-se manter a gravidez e o seguimento ambulatorial multidisciplinar por neurocirurgia e obstetrícia em consulta de alto risco. Optou-se por fazer cesárea eletiva às 39 semanas sob anestesia peridural lombar. No intraoperatório ocorreu um episódio de hipotensão rapidamente revertida com fenilefrina. O Índice de Apgar do recém-nascido foi de 10/10. O cateter peridural foi usado para analgesia pós-operatória, também sem intercorrências. CONCLUSÕES: São muito raros os casos publicados de abordagem anestésica de grávidas com MAV sintomáticas. Todas as decisões tomadas pela equipe multidisciplinar, desde optar por continuar a gravidez ao momento ideal para intervir na MAV, passando pelo tipo de anestesia e analgesia, foram ponderadas em função do risco de dano cerebral. Do ponto de vista anestésico, os autores enfatizam a necessidade de estabilidade hemodinâmica.

BACKGROUND AND OBJECTIVES: Subarachnoid hemorrhage (SAH) during pregnancy is a rare event, and about half the cases are due to arteriovenous malformations (AVM). The authors describe the anesthetic approach of a 39 week pregnant patient scheduled for cesarean section, with a history of SAH due to AVM at 22 week gestation. CASE REPORT: 39 week pregnant patient, healthy prior to pregnancy, with a history of SAH at 22 week gestation, manifested by headache, vomiting, and dizziness without loss of consciousness or other deficits on admission to the emergency room. Magnetic resonance imaging (MRI) revealed a left frontal AVM. After a short hospital stay for stabilization and diagnosis, the final medical decision was to maintain the pregnancy and a multidisciplinary follow-up by neurosurgery and high-risk obstetric consultation. An elective cesarean section was performed at 39 weeks under epidural anesthesia. During the intraoperative period, an episode of hypotension rapidly reversed with phenylephrine occurred. The newborn Apgar score was 10/10. An epidural catheter was used for postoperative analgesia, also uneventful. CONCLUSIONS: There are very few published cases of anesthetic approach for pregnant women with symptomatic AVM. All decisions made by the multidisciplinary team, from choosing to continue the pregnancy to the ideal time for AVM intervention and type of anesthesia and analgesia, were weighted according to the risk of brain damage. Regarding the anesthetic procedure, the authors emphasize the need for hemodynamic stability.

JUSTIFICATIVA Y OBJETIVOS: El aparecimiento de la hemorragia subaracnoidea (HSA) durante el embarazo es algo raro, siendo que aproximadamente la mitad se debe a malformaciones arteriovenosas (MAV). Los autores describen el abordaje anestésico de una embarazada de 39 semanas programada para cesárea, con un historial de HSA por MAV a las 22 semanas. RELATO DEL CASO: Embarazada de 39 semanas, sana antes del embarazo, con antecedentes de HSA a las 22 semanas de gestación que se manifestó por medio de cefaleas, vómitos y mareos, sin la pérdida de la consciencia u otros déficits a la hora de su entrada en el servicio de urgencia. La resonancia magnética (RM) arrojó MAV frontal izquierda. Después de un breve período de ingreso para la estabilización y el diagnóstico, se decidió mantener el embarazo y el acompañamiento ambulatorio multidisciplinario por neurocirugía y obstetricia en consulta de alto riesgo. Se optó por realizar la cesárea electiva a las 39 semanas bajo anestesia epidural lumbar. En el intraoperatorio ocurrió un episodio de hipotensión que fue rápidamente revertido con fenilefrina. El Índice de Apgar del recién nacido fue de 10/10. El catéter epidural fue usado para la analgesia postoperatoria, que también cursó sin intercurrencias. CONCLUSIONES: Son muy raros los casos publicados de abordaje anestésico de embarazadas con MAV sintomáticas. Todas las decisiones tomadas por el equipo multidisciplinario, desde optar por continuar con el embarazo, hasta el momento ideal para intervenir la MAV, pasando por el tipo de anestesia y analgesia, fueron sopesadas en función del riesgo de daño cerebral. Desde el punto de vista anestésico, los autores enfatizan la necesidad de estabilidad hemodinámica.

Occlusive hyperemia and normal pressure break through- physiopathology and management

Immediately following surgical excision of a cerebral AVM, even normal brain tissue surrounding the lesion is subject to hemorrhage, a phenomenon termed normal perfusion pressure breakthrough (NPPB) syndrome or occlusive hyperemia. According to this theory, arteries supplying cerebral AVMs become dilated and lose their capacity to dilate or constrict to autoregulate pressure. Acutely after removal of a cerebral AVM, excessive blood pressure in these arterial feeders can cause normal brain tissue to bleed. However, this theory still remains controversial.

Descolamento regmatogênico de retina na síndrome de Wyburn-Mason: relato de caso / Rhegmatogenous retinal detachment in Wyburn-Mason syndrome: case report

Wyburn-Mason is a rare vascular disorder, comprised of arteriovenous malformations (AVMs) of the midbrain and retina. It can cause visual symptoms depending on its localization and extension. Vitreous and intraretinal hemorrhage and neovascular glaucoma have been previously described. A case of rhegmatogenous retinal detachment in a patient with Wyburn-Mason syndrome is described. A 27 year-old woman previously diagnosed with Wyburn-Mason syndrome, sought attendance with sudden low vision in right eye 3 months before. She presented moderate vitreous hemorrhage and retinal detachment with a superior tear. She underwent a successful posterior vitrectomy with implantation of silicone oil, with reattachment of the retina. Rhegmatogenous retinal detachment in a patient with Wyburn-Mason syndrome has been not previously described in the literature. Vitrectomy in this case present challenges related to intraoperatory bleeding risk, to a posterior pole tear among AVMs and the difficulty of obtaining free retina for photocoagulation.

A síndrome de Wyburn-Mason é uma desordem vascular rara, caracterizada por malformações arteriovenosas (MAV) do mesencéfalo e retina. Pode causar sintomas visuais por sua localização e extensão. Hemorragia vítrea, intrarretiniana e glaucoma neovascular já foram descritos. Descreve-se um caso de descolamento regmatogênico de retina em uma paciente com síndrome de Wyburn-Mason. Uma paciente feminina de 27 anos com diagnóstico prévio de síndrome de Wyburn-Mason procurou atendimento com queixa de baixa acuidade visual súbita no olho direito há três meses. Apresentava hemorragia vítrea moderada e descolamento de retina com rotura superior. Foi submetida a vitrectomia posterior com implante intravítreo de óleo de silicone, com reaplicação da retina. Descolamento regmatogênico de retina em paciente com síndrome de Wyburn-Mason não havia sido previamente descrito na literatura. A vitrectomia nesse caso apresentou dificuldades relacionadas ao risco de sangramento intraoperatório, à presença de rotura no polo posterior entre as MAVs e à dificuldade de obtenção de retina livre para fotocoagulação.

Forensic appraisal of subarachnoid hemorrhage / 法医学杂志

Subarachnoid hemorrhage (SAH) can be classified as traumatic SAH or spontaneous SAH based on etiology. The traumatic SAH is the common manifestation of head injury and difficult to make a diagnosis in forensic investigation. Based on practical experiences of forensic investigation and relevant references, the main points (SAH identification, examination, death mechanism) were summarized in this article. For the understanding, we try to classify the traumatic SAH into three subtypes: brain injury associated SAH, traumatic rupture of basal cerebral vessels SAH and traumatic focal SAH.

Persistent trigeminal artery: angio-tomography and angio-magnetic resonance finding / Artéria trigeminal persistente: achado na angio-tomografia e angio-ressonância magnética

The trigeminal artery (TA) is the most common embryonic carotid-vertebrobasilar anastomosis to persist into adulthood. It typically extends from the internal carotid artery to the basilar artery. Persistent primitive arteries are usually found incidentally, but are often associated with vascular malformation, cerebral aneurysm and, in case of TA, with trigeminal neuralgia. We present one patient with TA as a cause of trigeminal neuralgia and in other three as an incidental finding, on TC and MR angiograms.

Artéria trigeminal (AT) é a anastomose carótido-vertebrobasilar mais comum a persistir na vida adulta. Tipicamente estende-se da artéria carótida interna até a artéria basilar. Artérias primitivas persistentes são usualmente um achado incidental, mas freqüentemente estão associadas a malformações vasculares, aneurismas e, no caso da AT, a neuralgia do trigêmio. Apresentamos um paciente com AT como causa de neuralgia do trigêmio e em outros três como um achado incidental, em angiogramas por tomomografia computadorizada e ressonância magnética.

Angioarchitecture and clinical presentation of brain arteriovenous malformations / Angioarquitetura e apresentação clínica de malformações arteriovenosas encefálicas

The purpose of this study was to correlate the angioarchitecture of brain arteriovenous malformations (AVM) with their clinical presentation. A total of 170 patients with AVM 78 males and 92 females, were studied. Univariate and multivariate analyses were conducted in order to test the associations between morphological features and clinical presentation. The most frequent clinical presentations at diagnosis were hemorrhage in 89 (52 percent) patients, headache in 79 (46 percent), focal neurological deficit in 54 (32 percent), and seizure in 52 (31 percent). According to the Spetzler-Martin classification, grade I was found in 15 patients, II in 49, III in 55, IV in 41, and grade V in 10 patients. AVM with small nidus size, single feeding artery and single draining vein were associated with hemorrhage. Hemorrhage was positively associated with Spetzler-Martin grade I and negatively with grade V. The association between seizure and large nidus size was positive, however negative with small nidus size.

O objetivo deste estudo foi correlacionar a angioarquitetura de malformações arteriovenosas encefálicas (MAV) com sua apresentação clínica. Foram estudados 170 pacientes portadores de MAV, sendo 78 do sexo feminino e 92 do masculino. Análises univariada e multivariada foram efetuadas para testar associações entre características morfológicas e quadro clínico. As principais formas de apresentação clínica no momento do diagnóstico incluíram hemorragia em 89 (52 por cento) pacientes, cefaléia em 79 (46 por cento), déficit focal em 54 (32 por cento) e convulsão em 52 (31 por cento). De acordo com a classificação de Spetzler e Martin, 15 pacientes tinham MAV grau I, 49 grau II, 55 grau III, 41 grau IV, e 10 grau V. MAV com nidus de tamanho pequeno, aferência e eferência únicas foram associados à hemorragia. Hemorragia foi positivamente associada com grau I e negativamente com grau V. A associação entre convulsão e nidus de tamanho grande foi positiva, porém negativa com nidus de tamanho pequeno.

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.

Anatomical-computerized tomography [CT scan] study on the arteriovenous malformations [AVMs] in the brain of Iraqi patients

Arteriovenous malformations [AVMs] of the brain are anomalies affecting different age groups of the population, and predisposing patients to significant neurological disability from stroke, epilepsy, or other clinical manifestations. Noninvasive modalities are revealing these lesions more frequently, and with more accuracy. Previous studies on Iraqi subjects with intracranial AVMs are scarce. The aim of the study is to correlate the CT findings of intracranial AVMs with the clinical presentations, anatomic locations, the size, and the predictable origin of the arteries feeding these lesions and their venous drainage. The charts and CT scans of fifty-four Iraqi patients with an AVM, 31 males and 23 females [male to female ratio 1.3: 1], ranging in age from 6-74 years [mean 37.7] who were seen at the Neurosurgical Hospital-Baghdad from October 1998 to August 2002 were reviewed. Supratentorial AVMs were present in 53 patients; one patient had a left cerebellar AVM. The lesion was solitary, and directly localized in a single lobe, with more in the right lobes [mainly the parietal and temporal] in the non-haemorrhagic lesions, and in the left lobes of the AVMs presented with haemorrhage. The diameter of the lesion varied from less than 2.5 cm to >/= 6.5 cm. AVM may present symptomatically at any age .The arterial and venous components of the AVM could be explained by the site of the lesion. The size of the AVM could be evaluated as a potential factor predicting future AVM haemorrhage risk. Long-term follow-up evaluation is necessary for assessing the natural history and prognosis for such lesions

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.

Importancia del drenaje venoso cortical en la historia natural de las fístulas durales / Importance of the cortical venous drainage in the natural history of dural arteriovenous fistulas

Dural arteriovenous fistulas are direct arteriovenous connections within the duramater either in the cranial or spinal region. They account for 10 to 15 percent of all intracranial arteriovenous malformations and they have been consistently reported since forty years. The clinical picture is variable with mild symptoms as tinnitus or secondary to ocular venous congestion, or severe ones, like neurologic symptoms or hemorrhage. They can present at any age but most of the patients are over 50 years old. The anatomy of this malformation is variable and sometimes difficult. Natural history is not completely known, therefore the best treatment is still a matter of debate. In order to achieve a favorable treatment, many publications have been focused in the classification of these malformations based on a relation between the anatomy and the natural history.

Ventricular arteriovenous malformation bleeding: a rare cause of headache in children. Case report

Cefaléia como queixa principal raramente ocorre num serviço de emergência pediátrica. Quando isso acontece, casos de cefaléia secundária que trazem risco de vida, tais como a hemorragia subaracnóide são raramente relatados. Apresentamos o caso de uma criança que apresentou cefaléia de forte intensidade associada a rigidez de nuca, sem outras anormalidades no exame físico. A angioressonância e angiografia digital evidenciaram malformação arteriovenosa na topografia do plexo coróide do ventrículo lateral direito, nutrida pela artéria coroidéia anterior, grau III na classificação de Spetzler (drenagem venosa profunda: 1; área de eloqüência: 0 e tamanho: 2). Nós discutimos as diferenças na apresentação clínica das malformações arteriovenosas encefálicas nas crianças e adultos.

Homonymous Hemianopia after Embolization of an Aneurysm-associated AVM Supplied by the Anterior Choroidal Artery

The primary objective for the treatment of arteriovenous malformations (AVM) of the brain is to reduce the risk of hemorrhage. The risk of hemorrhage is known to increase with the presence of an aneurysm associated with AVM. The purpose of this report is to describe the development of visual complications after the embolization of a hemorrhagic anterior choroidal artery feeding AVM with an associated aneurysm and to describe the possible causes.

Homonymous Hemianopia after Embolization of an Aneurysm-associated AVM Supplied by the Anterior Choroidal Artery

The primary objective for the treatment of arteriovenous malformations (AVM) of the brain is to reduce the risk of hemorrhage. The risk of hemorrhage is known to increase with the presence of an aneurysm associated with AVM. The purpose of this report is to describe the development of visual complications after the embolization of a hemorrhagic anterior choroidal artery feeding AVM with an associated aneurysm and to describe the possible causes.