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Introduction: Deep cavernomas of eloquent areas, located in the region of the basal nuclei and thalamus, account for 9 to 36% of these encephalic vascular malformations. Internal capsule cavernomas are particularly challenging, as they are surrounded by important projection fibers and their manipulation can lead to permanent deficits. To demonstrate through surgical cases that cavernomas of the internal capsule can be approached by frontal craniotomy, via the superior frontal sulcus, in a curative manner and with low morbidity. Methods: We presented two cases of cavernomas of the internal capsule operated, whose treatment was microsurgical resection via frontal craniotomy and access to the lesion via the superior frontal sulcus, described step-by-step. To elucidate the rationale behind the decision, we used preoperative images with an emphasis on the patients' tractography and the importance of comparing these images with anatomical specimens dissected in the neuroanatomy and microsurgery laboratory. Results: The two cases of internal capsule cavernomas, one in the anterior limb and the other in the posterior limb, were treated surgically via the superior frontal sulcus. Discussion: Both patients showed radiological cure and clinical improvement in the post-operative segment. The patient consented to the procedure and to the publication of his/her image. Treatment of internal capsule cavernomas via the superior frontal sulcus has proven to be a safe and effective option.
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Introduction: Developmental venous anomalies (DVAs) are considered variants of normal transmedullary veins. Their association with cavernous malformations is reported to increase the risk of hemorrhage. Expert consensus recommends meticulous planning with MR imaging, use of anatomical "safe zones", intraoperative monitoring of long tracts and cranial nerve nuclei, and preservation of the DVA as key to avoiding complications in brainstem cavernoma microsurgery. Symptomatic outflow restriction of DVA is rare, with the few reported cases in the literature restricted to DVAs in the supratentorial compartment. Case: We present a case report of the resection of a pontine cavernoma complicated by delayed outflow obstruction of the associated DVA. A female patient in her 20's presented with progressive left-sided hemisensory disturbance and mild hemiparesis. MRI revealed two pontine cavernomas associated with interconnected DVA and hematoma. The symptomatic cavernoma was resected via the infrafacial corridor. Despite the preservation of the DVA, the patient developed delayed deterioration secondary to venous hemorrhagic infarction. We discuss the imaging and surgical anatomy pertinent to brainstem cavernoma surgery, as well as the literature exploring the management of symptomatic infratentorial DVA occlusion. Conclusion: Delayed symptomatic pontine venous congestive edema is extremely rare following cavernoma surgery. DVA outflow restriction from a post-operative cavity, intraoperative manipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstem venous anatomy, and "safe entry zones" will further elucidate the etiology of and the efficacious treatment for this complication.
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Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels, lined by a single layer of endothelium. Acquired form of these lesions was related to previous radiation-therapy, and might be related to its pathophysiology, because the vascular endothelial growth factor has higher expression in the immature brain of the child. Consequently, although it occurs mainly in the paediatric population, it can happen in adults. We report a case of radiation-induced CCM in an adult patient presenting with aggressive behaviour and cerebral haemorrhage.
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Hemangioma Cavernoso do Sistema Nervoso Central , Adulto , Humanos , Criança , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico por imagem , Hemangioma Cavernoso do Sistema Nervoso Central/etiologia , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Fator A de Crescimento do Endotélio Vascular , Encéfalo/patologia , Hemorragia CerebralRESUMO
BACKGROUND: Hemichorea may point to a structural lesion in the contralateral basal ganglia with a large list of possible causes. Cavernous angioma may be rarely a possible cause for acute appearance of this movement disorder. CASE DESCRIPTION: We present a rare case of a 32-year-old female patient with hemichorea caused by a cavernoma (or cavernous angioma) in the contralateral insula and putamen with complete improvement of symptoms with surgical resection of the lesion. CONCLUSIONS: We believe that surgical resection of basal ganglia cavernomas may be feasible with minor risks and resolution of clinical symptoms in the immediate postoperative period.
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Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/cirurgia , Hemangioma Cavernoso/complicações , Hemangioma Cavernoso/cirurgia , Paresia/etiologia , Paresia/cirurgia , Adulto , Gânglios da Base/diagnóstico por imagem , Córtex Cerebral/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/terapia , Putamen/diagnóstico por imagemRESUMO
Microsurgery remains the gold standard treatment for intraventricular lesions, but it is not without limitations. The cerebral ventricles approach is considered a challenge for neurosurgeons because of their deep location and their intimate association with critical areas of the brain. It has been shown that direct endoscopic view is superior to magnetic resonance imaging (MRI) in identifying intraventricular structures and lesions. Also, during neuroendoscopy, there is no cerebrospinal fluid leak causing brain collapse as seen in microsurgery. Different surgical strategies should be shared and may improve and facilitate intraventricular lesions resection. Herein, a case of a successful microsurgical cavernoma resection with prior ventriculoscopy is described. A 28-year-old woman was admitted with intense holocranial headache. A mild stiff neck was observed. MRI showed a 3-cm lesion with heterogeneous signal intensity and no contrast enhancement suggestive of cavernoma. Initially, a ventriculoscopy was done to inspect the lesion and the surrounding ventricular structures. After that, a conversion technique from endoscopy to microsurgery was performed. The initial corticectomy was extended and the endoscope pathway was followed into the ventricular cavity. Then, the cavernoma was completely removed by microsurgical technique. No complications were observed. Control MRI showed total tumour removal. The patient remained asymptomatic with no recurrence after 1 year. This case illustrates a successful prior endoscopic approach followed by microsurgery as an alternative to a direct microsurgical approach for intraventricular cavernoma. A previous direct endoscopic view of the lesion and its relation with intraventricular structures may ensure a safe and complete resection by microsurgery.
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Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.
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Humanos , Feminino , Idoso , Glândula Pineal/cirurgia , Neoplasias Encefálicas/cirurgia , Hemangioma Cavernoso/cirurgia , Hidrocefalia/cirurgia , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/diagnóstico por imagem , Imageamento por Ressonância Magnética , Diagnóstico Diferencial , Hemangioma Cavernoso/patologia , Hemangioma Cavernoso/diagnóstico por imagemRESUMO
RESUMEN El síndrome de Millard-Gubler es considerado un síndrome protuberancial debido a una lesión pontina inferior y caracterizado, desde el punto de vista clínico, por parálisis facial y del VI par, ipsilateral a la lesión y parálisis braquiocrural contralateral; frecuentemente es de causa vascular, menos frecuente de causa traumática o por efecto de masa secundario a un tumor. Se realiza reporte de un caso de un paciente de 45 años de edad que acude al servicio hospitalario de la Fundación Centro Colombiano y Enfermedades Neurológicas FIRE con cuadro clínico caracterizado en pérdida de la fuerza muscular en hemicuerpo izquierdo, y déficit motor de la hemicara derecha, disartria y deterioro del estado de consciencia. La TAC de cráneo simple al ingreso evidenció sangrado extenso del tallo cerebral. De acuerdo con los hallazgos clínicos se concluye que el paciente presentó un síndrome de Millard-Gubler.
SUMMARY The Millard Gubler syndrome is considered a protuberant syndrome due to a lower pontine lesion and characterized, from the clinical point of view, by facial paralysis and VI pares, ipsilateral to the lesion and contralateral brachiocrural palsy; frequently it is of vascular cause, less frequent of traumatic cause or by effect of mass secondary to a tumor. A case report is presented of a 45-year-old patient who attends the hospital service of the Fundación Centro Colombiano y Neurológicas FIRE with a clinical picture characterized by loss of muscle strength in the left side of the body, and motor deficit of the right side of the face, dysarthria and deterioration of the state of consciousness. The simple skull CT on admission showed extensive brainstem bleeding. According to the clinical findings, it is concluded that the patient presented a Millard Gubler syndrome.
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Infartos do Tronco Encefálico , Paralisia Facial , Hemangioma CavernosoRESUMO
BACKGROUND: Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels lined by a single layer of endothelium. In contradistinction to arteriovenous malformations, these lesions do not have smooth muscle or elastin in their lining and they are angiographically occult, and the MRI is the most sensitive test for CCM detection. CCM are one of the most prevalent vascular malformations of the central nervous system, affecting about 0.4-0.6% of the general population. The main complication of this malformation is the risk of bleeding, which may cause neurological deficits that affect the quality of life (QoL) in patients. When symtomatic, they may be surgically treated for relieving the mass effect and seizures refractory to drug uses, hemorrhage and drug-refractory epilepsy. Patient-reported outcome (PRO) may be a strategy that can be used to evaluate QoL of CCM population and was used in a sample of non-operated patients. METHODS: An observational, cross-sectional analysis to evaluate the PRO using the SF-36 and EuroQol 5 dimensions (EQ-5D) questionnaires of QoL added to functional metrics using the Karnofsky Performance Status (KPS) in 49 patients not submitted to intervention and with long-term follow-up. RESULTS: During the 364 person-years of follow-up, there was an average of individual follow-up of 7.42 years. The mean age was 46.8 years (18-84) - 57% of them were female, 71% had superficial lesions, and 65% had the familial form. Comparisons of SF-36 dimensions with KPS graded <100 had a worse score only in terms of the pain (p = 0.04), vitality (p = 0.001), and general state of health (p = 0.03) domains. The domain mental health was worse in patients without surgical indication (p = 0.032). The functional capacity domain had the highest overall grading in the group. The EQ-5D dimensions of mobility (p = 0.03) and pain/discomfort (p = 0.001) were the ones with lower score compared to KPS <100. CONCLUSION: The study is the first to evaluate, with validated tools, the PRO of non-operated CCM patients and has demonstrated in a selected group of patients that it was possible to achieve long-term clinical stability, thereby maintaining QoL and functional neurological outcome.
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Tratamento Conservador , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico , Hemangioma Cavernoso do Sistema Nervoso Central/terapia , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Tratamento Conservador/efeitos adversos , Estudos Transversais , Feminino , Nível de Saúde , Hemangioma Cavernoso do Sistema Nervoso Central/fisiopatologia , Hemangioma Cavernoso do Sistema Nervoso Central/psicologia , Humanos , Avaliação de Estado de Karnofsky , Imageamento por Ressonância Magnética , Masculino , Saúde Mental , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Cavernous malformation (CM) is a vascular malformation found in the encephalic parenchyma, spinal cord, nerve roots, and extraneural tissue. CM in the trigeminal distribution is exquisitely uncommon and its biological behavior not completely understood. The clinical picture might be diverse, depending on the affected sector of the trigeminal architecture, and literature debating its pathobiology is scarce. CASE DESCRIPTION: We describe a case of 56-year-old woman who presented with left trigeminal neuralgia and a rapidly growing cavernous malformation of the entire distribution of the fifth nerve. The clinical picture evolved to a progressive gait ataxia and follow-up neuroimaging showed a large intracranial mass leading to a brainstem compression. After microsurgical resection, the mass proved to be a typical CM of the trigeminal root. CONCLUSION: We present an uncommonly aggressive progression of a CM of the trigeminal root, Gasserian ganglion, and cavernous sinus evolving to severe brainstem compression. The documentation of this unique case as well as its management is presented is discussed.
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Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico , Hemangioma Cavernoso do Sistema Nervoso Central/complicações , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico , Neuralgia do Trigêmeo/etiologia , Neoplasias Encefálicas/cirurgia , Feminino , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Humanos , Pessoa de Meia-Idade , Neuralgia do Trigêmeo/diagnóstico , Neuralgia do Trigêmeo/terapiaRESUMO
Introducción: Las lesiones del tronco cerebral son uno de los mayores desafíos neuroquirúrgicos. Los angiomas cavernosos de esta localización son lesiones de comportamiento agresivo, con alta morbi-mortalidad asociada, donde el neurocirujano tiene la posibilidad de curar al paciente pero con un nivel de riesgo que hacen que la oportunidad de la cirugía se mantenga en debate. Material y Método: Análisis retrospectivo de 8 casos de cavernomas de tronco operados entre los años 2009-2013 con sus características clínicas, quirúrgicas y de seguimiento. Se realiza además una revisión reflexiva sobre la evolución del manejo de estas lesiones y del estado del arte a nivel en el concierto internacional. Resultados: Los 8 casos presentaron evoluciones inmediatas y mediatas favorables tras la cirugía. No hubo mortalidad ni empeoramiento del status neurológico en relación al estado preoperatorio en ninguno de los 8 casos. Tres de los pacientes de la serie fueron operados tras caer en una condición clínica crítica y son los que registran los mayores déficits durante el seguimiento. El análisis de la evolución del manejo de este tipo de lesiones a nivel mundial revela una tendencia hacia el manejo quirúrgico precoz en lesiones sintomáticas. Conclusiones: Los resultados de esta serie así como la evolución del estado del arte permiten concluir que en pacientes sintomáticos una cirugía precoz ofrece en general mejores expectativas que intervenciones tardías para lesiones relativamente superficiales.
Introduction: Brainstem lesions are a major neurosurgical challenge. Cavernous angiomas of this location are lesions of aggressive behavior, with high morbidity and mortality associated, where the neurosurgeon has the possibility to cure the patient but with a level of risk that makes that the surgery timing remains under debate. Material and Methods: A retrospective analysis of 8 brainstem cavernomas cases operated in the period 2009-2013 is presented whit its clinical, surgical and follow-up characteristics. A thoughtful review of the evolution of the management of these lesions and the state of art in the international level is also carried out. Results: All 8 patients presented immediate and mediate favorable changes after surgery. There was no mortality or worsening of the neurological condition in relation to the preoperative status in any of the 8 cases. 3 patients in this series underwent surgery after falling in a critical condition and are those who have the highest deficit in the follow-up. The analysis of the evolution of the worldwide management of these injuries reveals a trend towards early surgical management in symptomatic lesions. Conclusions: The results of this series as well as the evolution of the state of the art let us conclude that in symptomatic patients an early surgery offers overall better expectations than later interventions in relatively superficial lesions.
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Humanos , Masculino , Adulto , Feminino , Pessoa de Meia-Idade , Hemangioma Cavernoso/cirurgia , Hemangioma Cavernoso/diagnóstico , Hemangioma Cavernoso/mortalidade , Procedimentos Cirúrgicos Operatórios/métodos , Tronco Encefálico/lesões , Estudos RetrospectivosRESUMO
La cavernomatosis cerebral (CC), única o múltiple, es la presencia de conductos vasculares distendidos, angiográficamente ocultos, por lo cual clásicamente eran hallazgos incidentales intraoperatorios o en autopsias. Actualmente la resonancia magnética (RM) cerebral, permite identificar éstas lesiones en un mayor número de pacientes. Las manifestaciones clínicas son hemorragias intracerebrales, crisis epilépticas y cefalea. Se reporta el caso de un varón de 55 años, sin antecedentes de importancia que ingresó por presentar cefalea y crisis de arresto del habla. El examen físico fue normal. Estudios tomográficos previos mostraron una lesión hemorrágica única, al realizarse una RM cerebral con protocolo T2-eco gradiente se evidencia múltiples imágenes compatibles con CC múltiple. Es el primer reporte de CC múltiple, entidad poco diagnosticada, que se debe sospechar en pacientes sin antecedentes de riesgo vascular que debutan con hemorragias intracerebrales, crisis epilépticas y/o cefaleas; constituyendo la RM cerebral en protocolo T2-eco gradiente, el estudio indicado para el diagnóstico.
Cerebral cavernomatosis (CC), single or multiple, is the presence of distended vascular channels, angiographically occult, for that, they are classically incidental intraoperative findings at autopsy. Currently the magnetic resonance imaging (MRI) let us identify these lesions in a larger number of patients. Clinical manifestations are intracerebral hemorrhages, seizures and headache. A male, 55, no history of significance is reported; he was admitted for headache and some months later presents speech arrest crisis. On physical examination, nothing unusual. Previous tomographic studies showed a hemorrhagic lesion, when the brain MRI with T2-eco gradient protocol was performed, multiple images observed, were compatible with multiple CC. This is the first report of multiple CC, unfrequent entity that should be suspected in patients with no history of vascular risk who present intracerebral hemorrhage, seizure and / or headache; being the brain MRI, the study indicated for diagnosis.
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BACKGROUND AND OBJECTIVES: Brain magnetic resonance is a major exam to evaluate thunderclap headache, after excluding subarachnoid hemorrhage. This study aimed at reporting a case of brainstem cavernous angioma (cavernoma) where clinical presentation and computerized tomography have suggested intraventricular hemorrhage. CASE REPORT: Female patient, 55 years, was referred to the hospital with a history of new headache 10 days ago. Pain onset was sudden, pressure-type, severe, located in the occipital region with irradiation throughout the head, followed by severe photophobia, nauseas, diplopia and blurred vision. Neurological evaluation has revealed anisocoria, complete ophthalmoplegia and right eyelid ptosis. Cranial CT has shown blood in the third ventricle. Conventional brain arteriography has not shown aneurysm, arteriovenous malformation or venous sinus thrombosis. At lumbar puncture, an opening water pressure of 45cm was found and liquor analysis was normal. Brain resonance has shown oval lesion (1.0x1.0x0.6cm) of exophytic aspect in the interpeduncular cistern and third ventricle, compatible with brainstem cavernoma. CONCLUSION: In this case, magnetic resonance was essential for the diagnosis, since routine exams (brain tomography, liquor puncture and arteriography) could not define it. Further studies are needed to explain how magnetic resonance impacts investigation. .
JUSTIFICATIVA E OBJETIVOS: A ressonância magnética cerebral é um exame importante na investigação da cefaleia em trovoada, após a exclusão de hemorragia subaracnoidea. O objetivo deste estudo foi relatar um caso de angioma cavernoso (cavernoma) no tronco cerebral, em que a apresentação clínica e tomografia computadorizada sugeriram uma hemorragia intraventricular. RELATO DO CASO: Paciente do gênero feminino, 55 anos, foi encaminhada ao hospital com uma história de cefaleia nova há 10 dias. A dor teve início súbito, do tipo pressão, de forte intensidade, localizada na região occipital com irradiação para toda a cabeça, acompanhada de fotofobia intensa, náuseas, diplopia e visão turva. O exame neurológico revelou anisocoria, oftalmoplegia completa e ptose palpebral à direita. Tomografia computadorizada de crânio mostrou sangue no terceiro ventrículo. Arteriografia cerebral convencional não apresentou aneurisma, malformação arteriovenosa ou trombose de seios venosos. Na punção lombar, uma pressão de 45cm de água de abertura foi encontrada e a análise do líquido cefalorraquidiano foi normal. A ressonância de crânio revelou lesão oval (1,0x1,0x0,6cm) de aspecto exofítica na cisterna interpeduncular e terceiro ventrículo compatível com cavernoma de tronco cerebral. CONCLUSÃO: No caso descrito, a ressonância magnética foi essencial para o diagnóstico, uma vez que os exames de rotina (tomografia de crânio, punção de líquor e arteriografia) não conseguiram defini-lo. Maiores estudos são necessários para esclarecer como a realização de ressonância magnética impacta a investigação. .
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Se presenta el caso clínico de un paciente de 57 años de edad, de la raza blanca, con cuadro clínico de compresión medular dorsal, por lo cual se le realizó una laminectomía de D5-D7, con resección de una lesión extradural, de color vino, redondeada, de 3x2x2 cm. Los resultados de la biopsia confirmaron el diagnóstico de una malformación angiomatosa (cavernoma). La cirugía es el tratamiento ideal para estos tipos de lesiones y la resonancia magnética de columna, un estudio útil para diagnosticar y tratar a los afectados.
The clinical case of a white 57 year-old patient is presented, with clinical pattern of dorsal medullary compression, reason why a laminectomy of D5-D7 was carried out, with resection of an extradural, rounded, wine colored lesion, of 3x2x2 cm. The results of the biopsy confirmed the diagnosis of an angiomatous malformation (cavernoma). Surgery is the ideal treatment for these types of lesions and the spine magnetic resonance is an useful study to diagnose and to treat those affected.
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Objetivo: Descrever a via transtentorial, por meio de craniotomia têmporo-occipital, para remoção de cavernoma pontino de localização dorsolateral, exemplificado pela apresentação de um caso. Conclusão: O acesso transtentorial demonstra-se útil e seguro para remoção de lesões localizadas nessa região da ponte.
Objective: To present the transtentorial route through a temporal-occipital craniotomy for surgical removal of a posterior-lateral pontine cavernomas, exemplified by the presentation of a case. Regional anatomy is discussed. Conclusion: The transtentorial approach may be used safely to remove lesions of highly located in the posterior-lateral region of the pons.
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Humanos , Feminino , Adulto , Hemangioma Cavernoso/cirurgia , Tronco Encefálico/cirurgiaRESUMO
Objetivo: Descrever a via pré-sigmoidea ampliada para acesso a lesões localizadas na região ventrolateral da ponte, exemplificado com a apresentação de um caso de cavernoma pontino nessa localização. Conclusão: Esse acesso, amplamente revisto pela literatura, é útil para remoção cirúrgica de cavernomas pontinos ventrolaterais.
Objective: To describe the pre-sigmoid transpetrosal approach to the ventrolateral pontine region, exemplified by the presentation of a case of cavernoma in this localization. Conclusion: This approach is useful to remove pontine ventrolateral cavernomas.
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Humanos , Feminino , Adulto Jovem , Hemangioma Cavernoso/cirurgia , Tronco Encefálico/cirurgiaRESUMO
Os autores apresentam um caso em que um angioma cavernoso, envolvendo o nervo óptico, comprometeu progressivamente a visão direita de um paciente do sexo masculino, de 29 anos de idade. O paciente foi operado e o anatomopatológico comprovou o diagnóstico. É feita a revisão da literatura, em que 18 casos foram previamente relatados, existindo o risco de hemorragia, acarretando a apoplexia quiasmática. A importância da ressonância magnética para o diagnóstico é confirmada. O diagnóstico precoce e a total remoção da lesão são o tratamento de escolha para essa lesão.
A case of a cavernous angioma of the optic nerve is presented and 18 previous literature reports were reviewed. The patient presented with gradually progressive right-sided visual disturbance. The surgicaland histological findings demonstrated a cavernous angioma which was totally removed, although, the visual deficit persisted. Review of previously reported cases revealed that cavernous malformations in the optic nerve or chiasma present haemorrhage more frequently than those in the brain. Early diagnosis with total excision is the treatment of choice for cavernous angioma of the optic chiasma.