Hemorragia intracraniana en un feto con trisomia 21 y rabdomiomas cardiacos: diagnostico diferencial de esclerosis tuberosa: estudio antenatal a propósito de un caso / Intracranian hemorrhage in a fetus with trisomy 21 and cardiac rhabdomyomas: differential diagnosis of tuberous sclerosis: antenatal study regarding a case

INTRODUCCIÓN: El Síndrome de Down (SD) es una de las aneuploidías más frecuentes. En Chile, la incidencia es de 2.2 por 1000 nv. La esclerosis tuberosa (ET) es una enfermedad genética autosómica dominante con una prevalencia de 1:600 a 1:10.000 nacidos vivos (nv) que se sospecha prenatalmente por la presencia de rabdomiomas cardiacos. Los tumores cardiacos fetales tienen una prevalencia de 1:10000 nv, los más prevalentes son los Rabdomiomas. El hallazgo de tumores intracraneanos son infrecuentes, dentro del diagnóstico diferencial es necesario descartar la Hemorragia Intraventricular (HIV). CASO CLÍNICO: Paciente de 29 años, M2, Derivada a nuestro centro a las 27+4 semanas para evaluación ecográfica. Entre los hallazgos se encuentran tumores intracardiacos en ventrículo derecho y marcadores blandos para alto riesgo de aneuploidía, por lo que se realiza cariotipo (amniocentesis genética: 47, XX+21). A las 32+0 semanas en una nueva evaluación presenta imagen hiperecogénica sugerente de tumor intracerebral. Se solicita resonancia magnética fetal que informa hemorragia intraventricular (HIV). El parto ocurre con un recién nacido de término, fenotipo concordante con Trisomía 21, ecocardiograma confirma dos tumores intracardiacos (Rabdomiomas) y ecografía cerebral confirma el diagnóstico de HIV Grado III derecho. Ante el diagnóstico diferencial de ET, se realiza ANGIO-TAC que resulta negativo para ET. DISCUSIÓN: En la evaluación ecográfica antenatal, la presencia de tumores intracardiacos asociados a tumor cerebral hace plantear el diagnóstico de una ET. El diagnóstico antenatal de tumores cerebrales vs HIV por ultrasonido es difícil. La resonancia es un examen complementario de gran ayuda, permitiendo un diagnóstico de certeza. La HIV fetal es un diagnóstico poco frecuente de diagnostico prenatal asociado a feto con trisomia 21.

INTRODUCTION: Down Syndrome (DS) is one of the most frequent aneuploidies. In our country its incidence is 2.2 every 1000 newborns. Tuberous sclerosis (TS) is a dominant autosomal genetic disease with a prevalence of 1:6000 to 1:10.000 newborns, this disease is suspected by the finding of cardiac rhabdomyomas. Rhabdomyomas are the most prevalent fetal heart tumors. Intracranial tumors are a rare prenatal finding in ultrasound the main differential diagnosis is Intraventricular Hemorrhage (IVH). The Objective of this paper is present a case report of a fetus with trisomy 21 plus rhabdomyomas and cranial tumors. CASE REPORT: 29 years old patient, referred for ultrasound at 27+4 week. Cardiac tumors and aneuploidy soft markers are found. Genetic amniocentesis is performed (Result: 47, XX+21). At 32+0 weeks ultrasound finding of intracranial tumor. Fetal MRI was performed which reports suspected IVH. Confirmed postnatally. Baby was delivered at term. Neonatal findings: Trisomy 21 phenotype, Echocardiogram with two cardiac tumors (Rhabdomyomas), neonatal brain ultrasound confirms Grade III - IVH. To rule out TS, an Angio-CT is performed which is negative for the disease. DISCUSSION: Cardiac Tumors associated to brain tumors in antenatal period make TS a possible diagnosis. Differentiate brain tumors and IVH by ultrasound is very difficult. MRI is a very helpful tool for an accurate diagnostic. IVH is a rare antenatal diagnosis. Not reported before in a baby with trisomy 21.

Bleeding risk of small intracranial aneurysms in a population treated in a reference center / Risco de sangramento de aneurismas intracranianos pequenos em uma população tratada em um centro de referência

ABSTRACT Large multicenter studies have shown that small intracranial aneurysms are associated with a minimal risk of bleeding. Nevertheless, other large series have shown that most ruptured aneurysms are, in fact, the smaller ones. In the present study, we questioned whether small aneurysms are indeed not dangerous. Methods: We enrolled 290 patients with newly-diagnosed aneurysms at our institution over a six-year period (43.7% ruptured). We performed multivariate analyses addressing epidemiological issues, cardiovascular diseases, and three angiographic parameters (largest aneurysm diameter, neck diameter and diameter of the nutrition vessel). Risk estimates were calculated using a logistic regression model. Aneurysm size parameters were stratified according to receiver operating characteristic (ROC) curves. Finally, we calculated odds ratios for rupture based on the ROC analysis. Results: The mean largest diameter for the ruptured versus unruptured groups was 13.3 ± 1.7 mm versus 22.2 ± 2.2 mm (p < 0.001). Multivariate analysis revealed a positive correlation between rupture and arterial hypertension (p < 0.001) and an inverse correlation with all three angiographic measurements (all p < 0.01). Aneurysms from the anterior cerebral artery bled more often (p < 0.05). According to the ROC curves, at the largest diameter of 15 mm, the sensitivity and specificity to predict rupture were 83% and 36%, respectively. Based on this stratification, we calculated the chance of rupture for aneurysms smaller than 15 mm as 46%, which dropped to 25% for larger aneurysms. Conclusion: In the population studied at our institution, small aneurysms were more prone to bleeding. Therefore, the need for intervention for small aneurysms should not be overlooked.

RESUMO Grandes estudos multicêntricos demostram que aneurismas intracranianos pequenos são associados a risco de sangramento mínimo. Outras grandes séries têm evidenciado que aneurismas rotos são em sua maioria os pequenos. Neste estudo questionamos até que ponto os aneurismas pequenos não são perigosos. Métodos: Avaliamos 290 novos casos de aneurismas tratados em nossa instituição durante 6 anos (43,7% rotos). Realizamos análises multivariadas com aspectos epidemiológicos dos pacientes, doenças cardiovasculares e três parâmetros angiográficos: maior diâmetro, diâmetro do colo e diâmetro do vaso nutridor do aneurisma. Estimativas de risco foram calculadas utilizando-se modelo de regressão logística. Parâmetros do tamanho aneurismático foram estratificados de acordo com curvas ROC. Também calculamos a razão de chances (odds ratios) de ruptura baseadas nas análises das curvas ROC. Resultados: O maior diâmetro médio para os grupos de aneurismas rotos e não-rotos foi 13.3 ± 1.7mm e 22.2 ± 2.2 (p < 0.001). Análises multivariadas revelaram uma correlação positiva entre ruptura aneurismática e hipertensão arterial (p < 0.001) e uma correlação inversa entre ruptura e as três medidas angiográficas (p < 0.01). Aneurismas da artéria cerebral anterior foram os que mais sangraram (p < 0.05). Análises das curvas ROC demonstram que no maior diâmetro de 15mm, a sensibilidade e especificidade para se predizer ruptura são de 83% e 36%. Baseando-se nessas estratificações, calculamos uma chance de ruptura para aneurismas menores de 15mm de 46% e de 25% para aneurismas maiores. Conclusão: Na população estudada, aneurismas pequenos são mais propensos a romper. Desta forma, a necessidade de intervenção para aneurismas pequenos não deve ser relevada.

Hemorragia intracraneal asociada a preeclampsia severa / Intracranial haemorrhage associated with severe preeclampsia

La hemorragia intracraneal constituye una patología poco frecuente del embarazo y asociada a pobres resultados materno-fetales. Dentro de sus causas se encuentra, la preeclampsia severa, la cual aumenta la mortalidad en estas pacientes. Se presenta el caso de una gestante que acude al servicio de urgencias con síntomas neurológicos e hipertensión arterial, diagnosticándose preeclampsia severa, con realización de cesárea de urgencia. La tomografía de cráneo mostró hemorragia temporal izquierda, requiriendo manejo en cuidados intensivos con posterior estabilización y alta médica. La hemorragia intracraneal requiere un alto índice de sospecha, monitoreo intensivo y manejo multidisciplinario.

Intracranial hemorrhage is a rare complication during pregnancy, it is associated with poor maternal and fetal outcomes. Cerebral haemorrhage caused by pre-eclampsia increases mortality in these patients. We present the case of a pregnant woman who consulted to the service of urgencies with neurological symptoms and hypertension. Severe preeclampsia was diagnosed. She underwent successful emergent cesarean section. The brain scan showed a left temporal hemorrhage. She required intensive care management with subsequent stabilization and discharged from the hospital. Intracranial hemorrhage requires a high index of suspicion, intensive monitoring, and a multidisciplinary approach.

Tratamento das hemorragias intracranianas espontâneas: o dilema continua / Treatment of Spontaneous Intracranial Hemorrhages: the Dilemma Continues

Introdução As doenças cerebrovasculares (DCV) são a principal causa de morte no Brasil, sendo um grande problema de saúde pública em todo o mundo. Métodos Revisão da literatura utilizando o banco de dados do MEDLINE. Buscados os termos "intracerebral" e "hemorrhage" presentes no título e no resumo publicados em qualquer data. Resultados As hemorragias intracranianas (HIC) acometem, principalmente, os lobos cerebrais, gânglios da base, tálamo, tronco cerebral (predominantemente a ponte) e cerebelo como resultado da ruptura de vasos cerebrais afetados pelos efeitos degenerativos da hipertensão arterial sistêmica (HAS) ou da angiopatia amiloide. O diagnóstico pode ser feito através da tomografia computadorizada de crânio (TCC), sendo auxiliado pela ressonância nuclear magnética (RNM) do encéfalo e a angiografia dos quatro vasos cerebrais na investigação etiológica. Tratamento: dividido em clínico e cirúrgico. Atualmente, não há consenso sobre a seleção do tipo de tratamento para pacientes com HIC, e esta decisão deve levar em consideração idade do paciente, estado neurológico, tamanho e profundidade do hematoma, presença de hidrocefalia e de efeito compressivo. Os estudos atuais mostram uma tendência de superioridade dos procedimentos cirúrgicos minimamente invasivos, commenor risco de lesão encefálica secundária decorrente do acesso cirúrgico ao hematoma. Conclusão A escolha da melhor estratégia para o tratamento das HIC permanece um desafio e ainda deve ser feita de forma individualizada.

Introduction Cerebrovascular Diseases are the major cause of death in Brazil and a public health issue in the world. Methods Review of the literature using the MEDLINE's data bank. We have searched the keywords "intracerebral" and "hemorrhages" in the title and abstract. Results Intracranial hemorrhages (ICH) affect, mainly, the cerebral lobes, basal ganglia, thalamus, brain stem and the cerebellum as a result of the rupture of diseased cerebral vessels by the effects of hypertension or amyloid angiopathy. Diagnosis can be done with the use of a non-contrast computed tomography (CT), magnetic resonance imaging (MRI) and cerebral angiogram (useful in investigation of the etiology). Treatment can be divided in clinical and surgical. This decision still should be taken considering individual features, such as patient's age and neurological status, hematoma's size and deep, time between ictus and the procedure, presence of hydrocephalus and compressive effects. The studies has shown a preference for the minimally invasive procedures, since the secondary brain lesions caused by the surgery tend to be less. Conclusion The choice of what would be the best strategy to treat the ICH is still a challenge and this decision should be taken individually.

Hemorrhagic intracranial distal (cortical) dissections: five cases and a literature review / Dissecções hemorrágicas intracranianas distais (corticais): cinco casos e revisão da literatura

Objective: To describe and analyze five cases of symptomatic intracranial spontaneous cortical dissections and a review of the literature relating to this disorder. Method: Retrospective analysis of patients undergoing investigation for intracranial hemorrhage. Data were compiled from the medical file and PACS of the Nancy University Hospital. Results: Of 350 patients who underwent investigation for spontaneous intracranial hemorrhage, five patients (four women) harbored intracranial distal dissections, with no evidence of infection, trauma, arterial hypertension, vasculitis. The patients age ranged from 35 to 77 years (mean age 50.8 years). No patient presented with a poor grade SAH (Hunt and Hess grade IV or V). All patients presented with headache. The localization of the vessel abnormality was equally distributed between the anterior and posterior circulation. All patients, except one, harbored an aneurysmal sac angiographically detectable. Conclusion: Dissecting aneurysms of the intracranial distal/cortical arteries are exceedingly rare vascular lesions that produce neurologic symptoms related to their topography and hemorrhagic or ischemic nature. In the aneurysmal forms, the treatment in an urgent fashion may be considered if we consider the risk of re-hemorrhage. The first-choice treatment is coil occlusion of the involved artery at the level of the dissection.

Objetivo: Descrever e analisar 5 casos de dissecção intracraniana de artérias distais espontâneas sintomáticas, com revisão da literatura. Métodos: Análise retrospectiva dos prontuários e imagens de tomografia, ressonância e angiografia. Os dados foram compilados do arquivo de imagens eletrônicas em formato DICOM através do sistema PACS do Hospital Universitário de Nancy, França. Resultados: De 350 pacientes investigados por hemorragia intracraniana espontânea, foram identificados 5 pacientes (4 do sexo feminino) com aneurismas intracranianos distais, onde foram descartadas as etiologias habituais (infecção, trauma, hipertensão arterial, vasculite). A média de idade foi de 50,8 anos, com mínima de 35 e máxima de 77 anos. Nenhum paciente estava com hemorragica de alto grau (Hunt-Hess grau 4 ou 5). Todos tiveram cefaléia súbita como sintoma inicial. A localização da anormalidade vascular foi igualmente distribuida entre a circulação anterior e posterior. Todos os pacientes, exceto um, apresentavam aneurisma angiograficamente detectável. Conclusão: Pequenas hemorragias intracranianas em vasos corticais são frequentemente encontradas, mas raramente investigadas. Aneurismas dissecantes das artérias distais/corticais são lesões extremamente raras que produzem sintomas relacionados a sua topografia e natureza isquêmica ou hemorrágica. A história natural destas lesões é pouco conhecida ou documentada. Aneurismas devem ser ocluidos preferencialmente com micromolas, evitando ressangramento.

Computed tomographic findings in non-traumatic hemorrhagic stroke.

OBJECTIVE: To analyze CT findings in hemorrhagic stroke patients correlation with clinical outcome and assess the interobserver agreement of hemorrhagic stroke identification on CT imaging. MATERIAL AND METHOD: CT imaging features of 131 cases and clinical data were verified and collected at Siriraj Hospital from Jan 2004 to Dec 2005 and retrospectively analyzed for type, location, mass effect, size of hemorrhage, intraventricular extension, initial level of consciousness (GCS), hospital length of stay and patient outcome. The percentages, predictive values, kappa were calculated. RESULTS: From all types of hemorrhagic stroke, intracerebral hemorrhage remains a common and devastating clinical problem. The most common site was the thalamus and basal ganglia. In the present study, the authors found that fifty-three cases (53/131 cases, 40.5%) with thalamic-ganglionic hemorrhage, nineteen cases (19/131 cases, 14.5%) in lobar hemorrhage, five cases (5/131 cases, 3.8%) in cerebellum, five cases (5/131 7 cases, 3.8%) in brainstem and eight cases (8/131 cases, 6.1%) occurred in multiple locations. There were twenty-five cases (25/131 cases, 19.1%) of subarachnoid hemorrhage, thirteen cases (13/131 cases, 9.9%) of subdural hemorrhage and three cases (3/131 cases, 2.3%) of intraventricular hemorrhage. Two variables on CT imaging, identified as significant as early mortality predictors, were hematoma volume more than 60 cm3, and presence of intraventricular hemorrhage extension (p < 0.05). The mass effect defined as midline and/or enlargement of contralateral ventricle was not significant (p = 0.067). The present study found concordance between CT brain interpretation by two neuroradiologists for the type of hemorrhagic stroke was very good, Kappa = 0.861 as well as for location was 0.866. CONCLUSION: CT imaging is an imaging instrument for early identification of hemorrhagic stroke patients and providing imaging evidence of high mortality risk.

Características de las hemorragias intracraneanas espontaneas en TC y RM / Characteristics of spontaneous intracranial hemorrhages on CT and MRI

The certain diagnosis of spontaneous intracranial hemorrhage represents a frequent challenge in radiologist practice, creating the need of being familiarized with this pathology . This article describes the neurobiological basis of the usual evolution of intracranial hematomas. It revises the imaging characteristics of its images, considering the variability of its appearance in computed tomography and magnetic resonance. Radiological signs are described and signs that help us in the distinction from benign to malign origin of hematomas. Finally, the frequent causes of spontaneous haemorrhage are described.

El diagnóstico certero de la hemorragia intracraneana espontánea representa un desafío frecuente en la práctica del radiólogo, por lo que debemos estar familiarizados con esta patología. Este artículo describe las bases neurobiológicas de la evolución natural de los hematomas intracraneanos. Revisa también las características de sus imágenes, considerando la variabilidad de su apariencia en tomografía computada (TC) y resonancia magnética (RM). Además, se describen los signos radiológicos que ayudan en la distinción de hematomas de origen benigno y maligno. Por último, se consideran las causas frecuentes de hemorragia intracraneana espontánea.

Feasibility and Utility of Transradial Cerebral Angiograpy: Experience during the Learning Period

OBJECTIVE: We wanted to present our experiences for performing transradial cerebral angiography during the learning period, and we also wanted to demonstrate this procedure's technical feasibility and utility in various clinical situations. MATERIALS AND METHODS: Thirty-two patients were enrolled in the study. All of them had unfavorable situations for performing transfemoral angiography, i.e., IV lines in the bilateral femoral vein, a phobia for groin puncture, decreased blood platelet counts, large hematoma or bruise, atherosclerosis in the bilateral femoral artery and the insistence of patients for choosing another procedure. After confirming the patency of the ulnar artery with a modified Allen's test and a pulse oximeter, the procedure was done using a 21-G micorpuncture set and 5-F Simon II catheters. After angiography, hemostasis was achieved with 1-2 minutes of manual compression and the subsequent application of a hospital-made wrist brace for two hours. The technical feasiblity and procedure-related immediate and delayed complications were evaluated. RESULTS: The procedure was successful in 30/32 patients (93.8%). Failure occurred in two patients; one patient had hypoplasia of the radial artery and one patient had vasospasm following multiple puncture trials for the radial artery. Transradial cerebral angiography was technically feasible without significant difficulties even though it was tried during the learning period. Pain in the forearm or arm developed in some patients during the procedures, but this was usually mild and transient. Procedure-related immediate complications included severe bruising in one patient and a small hematoma in one patient. Any clinically significant complication or delayed complication such as radial artery occlusion was not demonstrated in our series. CONCLUSION: Transradial cerebral angiography is a useful alternative for the patients who have unfavorable clinical situations or contraindications for performing transfemoral cerebral angiography. For the experienced neurointerventionalists, it seems that additional training for perfoming transradial cerebral angiography is not needed.