Remarkable Clinical Improvement Following Microsurgical Resection of Left Lingual Gyrus Cerebral Cavernous Malformation: A Case Report

Moyamoya disease is a chronic and unusual cerebrovascular disorder characterized by progressive stenosis and occlusion of the distal portions of internal carotid arteries and its main branches within the circle of Willis. Posterior circulation (vertebral and basilar arteries) may also be affected; however, this presentation is uncommon. As well as stenosis of the terminal portion of intracranial arteries, it is seen the development of a network of collateral vessels abnormally dilated at the base of the brain with an aspect of a "puff of smoke," whose term in Japanese is described as "moyamoya." The present study aims to report two consecutive cases of patients who presented to our service with different clinical manifestations. Further investigation with digital subtraction angiography showed a moyamoya pattern.

Importancia del diagnóstico histopatológico en el tratamiento de las lesiones del tronco cerebral en adultos / Relevance of histopathological diagnosis in the treatment of brainstem lesions in adults

Los tumores del tronco cerebral son infrecuentes en la población adulta. Las controversias surgen cuando se considera la necesidad de confirmar el diagnóstico histopatológico en esta área elocuente del cerebro, balanceando los beneficios de obtener un diagnóstico certero y las desventajas de los procedimientos invasivos. Existen escasas publicaciones acerca de su tratamiento quirúrgico en adultos, todas series pequeñas analizadas retrospectivamente. Presentamos nuestra experiencia con el propósito de contribuir al proceso de toma de decisiones. Diez de 13 pacientes fueron intervenidos. Las lesiones se clasificaron en focales (n:7), infiltrativa difusa (n:1), tectal (1) y exofítica (1). El estado neurológico según la escala Karnofsky Performance Status fue ≥ 70 en 6 casos y < 70 en 7. Las muestras fueron obtenidas mediante abordaje microquirúrgico directo o por biopsia estereotáctica. Los hallazgos histopatológicos fueron confirmados en todos los casos: astrocitoma pilocítico (n:1), glioma de bajo grado (n:1), glioblastoma (n:1), hemangioblastoma celular (n:1), subependimoma (n:1), disgerminoma (n:1), y lesiones pseudotumorales (n:4, 3 cavernomas, 1 pseudotumor inflamatorio). La amplia variedad de hallazgos patológicos en esta localización en adultos exige una precisa definición histopatológica, que no solo determina la terapéutica adecuada sino que también previene las consecuencias potencialmente catastróficoas de los tratamientos empíricos.

Brainstem tumors are uncommon beyond childhood. Controversies arise regarding the need of histological diagnosis in this eloquent area of the brain, weighting the benefits of a reliable diagnosis against the disadvantages of invasive procedures. There are scant publications about the surgical management of brainstem tumors in adults, all of them involving small retrospective cohorts. We are reporting our experience with the aim of contributing to the decision making process. Out of a series of 13 patients, 10 were approached surgically. According to Guillamo´s classification the lesions were: focal (n:7), diffuse infiltrative (n:1), tectal (n:1), and exophytic (n:1). According to the Karnofsky Performance Status scale, the neurological status was ≥ 70 in 6 cases and < 70 in 7. Histopathology was confirmed in all 10 treated cases and the samples were obtained by a direct microsurgical approach or by stereotactic biopsy. Histopathological findings were: pilocytic astrocytoma (n:1), low grade glioma (n:1), glioblastoma (n:1), cellular haemangioblastoma (n:1), subependimoma (n:1), pseudotumoral lesions (n:4; 3 cavernomas, 1 inflammatory pseudotumor), and disgerminoma (n:1). As a broad variety of pathologies could be found in this brain localization, an accurate histopathological definition can not only determine the adequate therapy, but also avoid the disastrous consequences of empiric treatments.

Intracranial cavernous malformation in children: a single-centered experience with 30 consecutive cases / Angiomas cavernosos intracranianos em crianças: experiência de um único centro em 30 casos consecutivos

Objectives: To determine the clinical presentation and treatment outcome of pediatric intracranial cavernous malformation (CM) in a single-centered institution. Methods: Clinical data review of 30 patients under 18 years-old who had undergone surgery for cavernous malformation from January 1993 to December 2011. Results: The Study Group included 18 males and 12 females (mean age: 8.7 years-old). Symptoms at presentation were seizures (16/30, 53.3%), headache (15/30, 50.0%), and focal neurological deficits (11/30, 36.6%). Multiple cavernous malformations were found in 5/30 (16.6%). According to location, patients were classified in groups: (G1) brain-steam in 5/30 (16.6%), (G2) cerebellum in 2/30 (6.6%), (G3) supratentorial associated with seizures in 16/30 (53.3%), and (G4) supratentorial without seizures in 7/30 (23.3%). Surgical resection was performed in 26 out of 30 (86.6%) patients. The mean follow-up period was 4.1 years. Of 15 children followed-up with preoperative seizures, all were rendered seizure-free after surgery. Conclusions: For symptomatic solitary cavernous malformation, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. For multiple cavernous malformation or asymptomatic patients, the treatment modalities must be cautiously considered. .

Objetivos: Determinar a apresentação clínica e o acompanhamento do tratamento em crianças com angioma cavernoso intracraniano numa única instituição. Métodos: Revisão de dados clínicos de 30 pacientes menores de 18 anos com que passaram por uma cirurgia de angioma cavernoso intracraniano entre janeiro de 1993 a dezembro de 2011. Resultados: O grupo de estudo incluiu 18 sujeitos masculinos e 12 femininos (idade média: 8,7 anos). Os sintomas iniciais eram convulsões (16/30, 53,3%), cefaleia (15/30, 50,0%) e déficits neurológicos focais (11/30, 36,6%). Havia angiomas cavernosos intracranianos múltiplos em 5 de 30 (16.6%). A classificação foi feita em grupos de acordo com a localização: (G1) tronco cerebral em 5/30 (16,6%); (G2) cerebelo em 2/30 (6,6%); (G3) supratentoriais associados a convulsões em 16/30 (53,3%) e (G4) supratentoriais sem convulsões em 7/30 (23,3%). Ressecção cirúrgica foi realizada em 26 de 30 (86,6%) pacientes, com seguimento médio de 4,1 anos. De 15 crianças com convulsões pré-operatórias, todas ficaram livres das crises após a cirurgia. Conclusões: Para angioma cavernoso intracraniano solitário e sintomático, o tratamento de escolha é excisão microcirúrgica total precedida de avaliação funcional e anatômica meticulosa. Para angiomas cavernosos intracranianos múltiplos ou pacientes assintomáticos, as modalidades terapêuticas devem ser consideradas cautelosamente. .

Brainstem cavernomas: a surgical challenge / Cavernomas de tronco: desafio cirúrgico

Objective: The authors show their experience with brainstem cavernomas, comparing their data with the ones of a literature review. Methods: From 1998 to 2009, 13 patients harboring brainstem cavernomas underwent surgical resection. All plain films, medical records and images were reviewed in order to sample the most important data regarding epidemiology, clinical picture, radiological findings and surgical outcomes, as well as main complications. Results: The mean age was 42.4 years (ranging from 19 to 70). No predominant gender: male-to-female ratio, 6:7. Pontine cases were more frequent. Magnetic resonance imaging was used as the imaging method to diagnose cavernomas in all cases. The mean follow-up was 71.3 months (range of 1 to 138 months). Clinical presentation was a single cranial nerve deficit, VIII paresis, tinnitus and hearing loss (69.2%). All 13 patients underwent resection of the symptomatic brainstem cavernoma. Complete removal was accomplished in 11 patients. Morbidity and mortality were 15.3 and 7.6%, respectively. Conclusions: Cavernomas can be resected safely with optimal surgical approach (feasible entry zone) and microsurgical techniques, and the goal is to remove all lesions with no cranial nerves impairment.

Objetivo: Os autores mostram sua experiência com cavernomas de tronco cerebral, comparando seus dados com os de uma revisão da literatura. Métodos: De 1998 a 2009, 13 pacientes com cavernoma de tronco cerebral foram submetidos a ressecção cirúrgica. Todos os filmes, prontuários e imagens foram revisados para exposição dos dados mais importantes, como epidemiologia, detalhes clínicos, achados radiológicos e resultados cirúrgicos, bem como as principais complicações. Resultados: A média de idade foi de 42,4 anos (variação de 19 a 70). Não houve predominância de gênero na taxa masculino versus feminino, 6:7. Os casos pontinos foram os mais frequentes. Ressonância nuclear magnética foi o método de imagem para o diagnóstico de cavernomas em todos os casos. A média do acompanhamento foi de 71,3 meses (variação de 1 a 138 meses). A apresentação clínica mais frequente foi a paresia do VIII nervo craniano, tinitus e perda auditiva (69,2%). Todos os 13 pacientes com cavernomas de tronco sintomáticos foram submetidos à ressecção cirúrgica. A remoção total foi realizada em 11 pacientes. A morbidade e a mortalidade foram de 15,3 e 7,6%, respectivamente. Conclusão: Os cavernomas podem ser seguramente ressecados por meio de acessos cirúrgicos ideais (zonas de entrada seguras) e técnicas de microcirurgia, sendo que o objetivo é remover toda a lesão sem o comprometimento dos nervos cranianos.

Application of Intraoperative Ultrasonography for Guiding Microneurosurgical Resection of Small Subcortical Lesions

OBJECTIVE: We wanted to evaluate the clinical value of intraoperative ultrasonography for real-time guidance when performing microneurosurgical resection of small subcortical lesions. MATERIALS AND METHODS: Fifty-two patients with small subcortical lesions were involved in this study. The pathological diagnoses were cavernous hemangioma in 25 cases, cerebral glioma in eight cases, abscess in eight cases, small inflammatory lesion in five cases, brain parasite infection in four cases and the presence of an intracranial foreign body in two cases. An ultrasonic probe was sterilized and lightly placed on the surface of the brain during the operation. The location, extent, characteristics and adjacent tissue of the lesion were observed by high frequency ultrasonography during the operation. RESULTS: All the lesions were located in the cortex and their mean size was 1.3 +/- 0.2 cm. Intraoperative ultrasonography accurately located all the small subcortical lesions, and so the neurosurgeon could provide appropriate treatment. Different lesion pathologies presented with different ultrasonic appearances. Cavernous hemangioma exhibited irregular shapes with distinct margins and it was mildly hyperechoic or hyperechoic. The majority of the cerebral gliomas displayed irregular shapes with indistinct margins, and they often showed cystic and solid mixed echoes. Postoperative imaging identified that the lesions had completely disappeared, and the original symptoms of all the patients were significantly alleviated. CONCLUSION: Intraoperative ultrasonography can help accurately locate small subcortical lesions and it is helpful for selecting the proper approach and guiding thorough resection of these lesions.

Spinal intramedullary cavernous hemangioma

The spinal cord can be involved in a variety of disease processes. These can be congenital or acquired. An acute onset of symptoms usually allows a defined set of causes to be considered including trauma, ruptured vascular anomalies, demyelination, and myelitis. Intramedullary cavernous hemangioma of the spinal cord is a congenital or acquired vascular malformation, and one of the rare causes of hematomyelia. We present such a case, and discuss the symptoms, diagnosis, and suggested best treatment options based on a review of present day literature

Cavernomas: conduta no serviço de neurocirurgia da Santa Casa de Belo Horizonte / Cavernous malformations: surgical management in Belo Horizonte Santa Casa Hospital

A melhor compreensão da história natural dos cavernomas, o aprimoramento dos métodos diagnósticos e das técnicas microcirúrgicas tornaram possíveis as seguintes condutas: tratamento conservador, radiocirurgia e ressecção cirúrgica. Apresenta-se revisão de 33 pacientes operados no serviço de Neurocirurgia da Santa Casa de Belo Horizonte, no período de 1992 a 2001. Cavernomas corticais e subcorticais que se manifestaram por crises convulsivas de difícil controle (57,5 por cento) ou principalmente por hemorragia (15,1 por cento) foram tratados cirurgicamente. As lesões profundas (gânglios basais, tálamo e tronco encefálico) somaram 27,7 por cento. Estas, atualmente, só devem ser operadas quando estão próximas à superfície ependimária ou pial. Indicou-se ainda a ressecção de lesões medulares (5,5 por cento) e cerebrais profundas, que apresentaram déficit focal progressivo(13,8 por cento) ou episódios recorrentes de hemorragia(13,8 por cento). As lesões pequenas e profundas que não apresentam hemorragia devem ser tratadas conservadoramente.Não existe, no momento, evidência de resultados favoráveis com a radiocirurgia.

Intraventricular cavernoma

Intraventricular cavernoma is rare; only 45 cases have been reported in the literature. Magnetic resonance imaging has wildly contributed in the diagnosis and the surgical approach. Case file: We report a 45-year-old man presenting with syndrome of intracranial hypertesion and retrograde amnesia for one year. CT scan and MRI revealed an intraventricular tumour, which was responsible for active hydrocephalus. A ventriculo-peritoneal shunt was performed and a stereotactic biopsy allowed the diagnosis of cavernoma. Post operative follow-up was uneventful, the patient rests asymptomatic. Ventricular cavernoma is rare and should be suspected in atypical localisations in order to chosse the best surgical approach. The author attack the attention to that rare localisation of cavernoma and for its hemorrhagic risles

Manejo neuroquirúrgico de los cavernomas del sistema nervioso central / Neurosurgical treatment of central nervous system cavernous angiomas

Los cavernomas o angiomas cavernosos son malformaciones hamartomatosas vasculares benignas, en su mayoría encefálicas y supratentoriales, con prevalencia descrita entre el 0,1 y el 4 por ciento de la población general, de presentación esporádica o familiar. El presente estudio analiza los aspectos epidemiológicos, clínicos y pronósticos de los casos tratados en el Instituto de Neurocirugía Asenjo durante el año 2000. Para ello se recopiló información de 13 casos operados desde las fichas médicas y de los protocolos operatorios, registrándolos en una matriz Excel® para su análisis. Los resultados permiten concluir que el cuadro clínico de presentación es de tipo comicial en la mayoría de los pacientes de nuestra serie. La cirugía se plantea sólo en los casos sintomáticos. En nuestro centro las cifras de morbimortalidad son similares a las presentadas en la literatura internacional. Las lesiones profundas son un desafío por la dificultad técnica de los accesos y porque están asociadas a mayores complicaciones.