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.

Multiple Cerebral Cavernous Malformations

No abstract available.

Synchronous Orofacial Granulomatosis in a Patient with Brain Cavernous Hemangioma

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.

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.

Surgical Approaches for Symptomatic Cerebral Cavernous Malformations of the Thalamus and Brainstem

OBJECTIVE: Surgical resection of thalamic and brainstem cerebral cavernous malformations (CCMs) is associated with significant operative morbidity, but it may be outweighed, in some cases, by the neurological damage from recurrent hemorrhage in these eloquent areas. The goals of this retrospective cohort study are to describe the technical nuances of surgical approaches and determine the postoperative outcomes for CCMs of the thalamus and brainstem. MATERIALS AND METHODS: We reviewed an institutional database of patients harboring thalamic or brainstem CCMs, who underwent surgical resection from 2010 to 2014. The baseline and follow-up neuroimaging and clinical findings of each patient and the operative details of each case were evaluated. RESULTS: A total of eight patients, including two with thalamic and six with brainstem CCMs, were included in the study cohort. All patients had progressive neurological deterioration from recurrent CCM hemorrhage, and the median modified Rankin Scale (mRS) at presentation was 3. The median CCM maximum diameter and volume were 1.7 cm and 1.8 cm³, respectively. The thalamic CCMs were resected using the anterior transcallosal transchoroidal and supracerebellar infratentorial approaches each in one case (13%). The brainstem CCMs were resected using the retrosigmoid and suboccipital trans-cerebellomedullary fissure approaches each in three cases (38%). After a median follow-up of 11.5 months, all patients were neurologically stable or improved, with a median mRS of 2. The rate of functional independence (mRS 0-2) was 63%. CONCLUSION: Microneurosurgical techniques and approaches can be safely and effectively employed for the management of thalamic and brainstem CCMs in appropriately selected patients.

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management / Neurocisticose, cavernoma cerebral familiar e calcificações intracranianas: diagnóstico diferencial e acompanhamento adequado

ABSTRACT Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.

RESUMO A neurocisticercose (NCC) é um importante problema endêmico de saúde pública em algumas áreas do mundo, sendo epilepsia sua manifestação clínica mais comum. Múltiplas lesões intracranianas, geralmente com calcificações visualizadas em tomografia computorizada de crânio, são interpretadas como um dos critérios diagnósticos na fase crônica da doença. A ressonância magnética é o melhor teste de imagem para identificar a doença em diferentes estágios de sua forma cística mas apresenta limitações para demonstrar lesões calcificadas. Malformações cavernosas cerebrais, ou cavernomas, são malformações vasculares comuns ao sistema nervoso e epilepsia é também a sua forma mais frequente de apresentação. Na sua forma familiar cavernomas apresentam-se tipicamente com múltiplas lesões encefálicas e, frequentemente, com focos de calcificações na TC. Em alguns países, e determinadas regiões geográficas, onde neurocisticercose é endêmica, a neuroimagem mais usada para diagnóstico é a TC de crânio. Nesse contexto torna-se importante estabelecer bases para o diagnóstico diferencial entre as duas doenças, devido às diferentes formas de acompanhamento e tratamento adequado.

Two cases of familial cerebral cavernous malformation caused by mutations in the CCM1 gene / 소아과

Cerebral cavernous malformation (CCM) is a vascular malformation characterized by abnormally enlarged capillary cavities without any intervening neural tissue. We report 2 cases of familial CCMs diagnosed with the CCM1 mutation by using a genetic assay. A 5-year-old boy presented with headache, vomiting, and seizure-like movements. Brain magnetic resonance imaging (MRI) revealed multiple CCM lesions in the cerebral hemispheres. Subsequent mutation analysis of his father and other family members revealed c.940_943 del (p.Val314 Asn315delinsThrfsX3) mutations of the CCM1 gene. A 10-month-old boy who presented with seizure-like movements was reported to have had no perinatal event. His aunt was diagnosed with cerebral angioma. Brain and spine MRI revealed multiple angiomas in the cerebral hemisphere and thoracic spinal cord. Mutation analysis of his father was normal, although that of the patient and his mother revealed c.535C>T (p.Arg179X) mutations of the CCM1 gene. Based on these studies, we suggest that when a child with a familial history of CCMs exhibits neurological symptoms, the physician should suspect familial CCMs and consider brain imaging or a genetic assay.

Systemic Expression of Vascular Endothelial Growth Factor in Patients with Cerebral Cavernous Malformation Treated by Stereotactic Radiosurgery

OBJECTIVE: Increased expression of angiogenic factors, such as vascular endothelial growth factor (VEGF), is associated with the pathogenesis of cerebral cavernous malformations (CCMs). The purpose of this study was to investigate plasma levels of VEGF in normal subjects and in patients with CCM and to evaluate change in these levels following stereotactic radiosurgery (SRS). METHODS: Peripheral venous blood was collected from 6 patients with CCM before SRS using Gamma Knife and at the 1 week, 1 month, 3month, and 6 month follow-up visits. Plasma VEGF levels were measured using commercially available enzyme-linked immunosorbent assay kits. Peripheral blood samples were obtained from 10 healthy volunteers as controls. RESULTS: Mean plasma VEGF level of 41.9 pg/mL (range, 11.7–114.9 pg/mL) in patients with CCM at baseline was higher than that of the healthy controls (29.3 pg/mL, range, 9.2–64.3 pg/mL), without significant differences between CCM patients and controls (p=0.828). Plasma VEGF level following SRS dropped to 24.6 pg/mL after 1 week, and decreased to 18.5 pg/mL after 1 month, then increased to 24.3 pg/mL after 3 months, and 32.6 pg/mL after 6 months. Two patients suffering from rebleeding after SRS showed a higher level of VEGF at 6 months after SRS than their pretreatment level. CONCLUSION: Plasma VEGF levels in patients with CCM were elevated over controls at baseline, and decreased from baseline to 1 month after SRS and increased further for up to 6 months. Theses results indicated that anti-angiogenic effect of SRS might play a role in the treatment of CCMs.

Treatment and outcome of epileptogenic temporal cavernous malformations / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The aim of this study is to explore the treatment and outcome of epileptogenic temporal lobe cavernous malformations (CMs).</p><p><b>METHODS</b>We analyzed retrospectively the profiles of 52 patients diagnosed as temporal lobe CMs associated with epilepsy. Among the 52 cases, 11 underwent a direct resection of CM along with the adjacent zone of hemosiderin rim without electrocorticogram (ECoG) monitoring while the other 41 cases had operations under the guidance of ECoG. Forty-six patients were treated by lesionectomy + hemosiderin rim while the other six were treated by lesionectomy + hemosiderin rim along with extended epileptogenic zone resection. The locations of lesions, the duration of illness, the manifestation, the excision ranges and the outcomes of postoperative follow-up were analyzed, respectively.</p><p><b>RESULTS</b>All of the 52 patients were treated by microsurgery. There was no neurological deficit through the long-term follow-up. Outcomes of seizure control are as follows: 42 patients (80.8%) belong to Engel Class I, 5 patients (9.6%) belong to Engel Class II, 3 patients (5.8%) belong to Engel Class III and 2 patients (3.8%) belong to Engel Class IV.</p><p><b>CONCLUSION</b>Patients with epilepsy caused by temporal CMs should be treated as early as possible. Resection of the lesion and the surrounding hemosiderin zone is necessary. Moreover, an extended excision of epileptogenic cortex or cerebral lobes is needed to achieve a better prognosis if the ECoG indicates the existence of an extra epilepsy onset origin outside the lesion itself.</p>

Unusual Presentation of Cerebral Cavernous Malformation

Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage.

Manifestaciones imaginológicas de las malformaciones cavernosas del sistema nervioso central / Imaging Manifestations of Cavernous Malformations of the Central Nervous System

El propósito de esta revisión es resaltar la epidemiología, presentación clínica y características imaginológicas de las malformaciones cavernosas del sistema nervioso central, especialmente en sus diferentes localizaciones, con el objetivo de brindarle al radiólogo claves para el diagnóstico de esta patología potencialmente curable. De las bases de datos de nuestras instituciones recolectamos casos de malformaciones cavernosas. Las imágenes de TC, MR y angiografía fueron evaluadas, y el tamaño, la localización, las características imaginológicas y los hallazgos asociados fueron registrados. Adicionalmente, se revisó la literatura científica pertinente a este tema para proveer una manera práctica de hacer una aproximación diagnóstica de esta malformación. Las malformaciones cavernosas son lesiones vasculares congénitas compuestas por vasos sinusoidales que forman una masa compacta. Pueden ocurrir en cualquier sitio del encéfalo y de la médula espinal. Imaginológicamente pueden presentar ciertas características alarmantes que pueden simular otras patologías más agresivas e incluso malignas. El conocimiento de los hallazgos característicos según su localización es esencial para evitar tratamientos innecesarios. Las malformaciones cavernosas del SNC pueden presentar ciertos rasgos que se sobreponen con patologías más malignas. Complicaciones asociadas como son la hemorragia y el edema perilesional pueden afectar aún más el diagnóstico adecuado. Por esta razón, es indispensable que el radiólogo conozca el comportamiento imaginológico de estas lesiones.

The purpose of this revision is to highlight the demographics, clinical presentation, and imaging features of cavernous malformations (CA) of the central nervous system, especially in its different locations, with the purpose of providing the radiologist clues regarding the diagnosis of this potentially curable pathology. We collected cases of cavernous malformations from the databases at our institutions. CT, MR and angiographic studies were evaluated and lesion size, location, imaging characteristics, multiplicity, and associated findings were recorded. Additionally, the scientific literature pertinent to the subject was reviewed in a practical manner in order to provide a practical manner of making a diagnostic approach of this malformation. Cavernous malformations are congenital vascular lesions composed of sinusoid- type blood vessels that assemble into a compact mass. They may occur in any location in the brain and in the spinal cord. In terms of imaging, they may present certain alarming characteristics that may mimic more aggressive or malignant entities. Associated complications such as hemorrhage and perilesional edema may affect adequate diagnosis at an ever larger degree. For this reason, it is essential that the radiologist is familiar with the imaging behavior of these lesions.

Microsurgical Treatment and Outcome of Pediatric Supratentorial Cerebral Cavernous Malformation

OBJECTIVE: The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. METHODS: Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. RESULTS: The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. CONCLUSION: With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.

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. .

Features of a Chinese family with cerebral cavernous malformation induced by a novel CCM1 gene mutation / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Familial cerebral cavernous malformations (CCMs), characterized by hemorrhagic stroke, recurrent headache and epilepsy, are congenital vascular anomalies of the central nervous system. Familial CCMs is an autosomal dominant inherited disorder and three CCM genes have been identified. We report a Chinese family with CCMs and intend to explore clinical, pathological, magnetic resonance imaging (MRI) features and pathogenic gene mutation of this family.</p><p><b>METHODS</b>Totally 25 family members underwent brain MRI examination and clinical check. Two patients with surgical indications had surgical treatment and the specimens were subjected to histopathological and microstructural examination. In addition, polymerase chain reaction (PCR) and direct sequencing were performed with genomic DNA extracted from 25 family members' blood samples for mutation detection.</p><p><b>RESULTS</b>Brain MRI identified abnormal results in seven family members. All of them had multiple intracranial lesions and four cases had skin cavernous hemangioma. T2-weighted sequence showed that the lesions were typically characterized by an area of mixed signal intensity. Gradient-echo (GRE) sequence was more sensitive to find micro-cavernous hemangiomas. There was a wide range in the clinical manifestations as well as the age of onset in the family. The youngest patient was an 8-year-old boy with least intracranial lesions. Histopathological and microstructural examination showed that CCMs were typically discrete multi-sublobes of berry-like lesions, with hemorrhage in various stages of illness evolution. They were formed by abnormally enlarged sinusoids and the thin basement membranes. A novel T deletion mutation in exon 14 of CCM1 gene was identified by mutation detection in the seven patients. But unaffected members and healthy controls did not carry this mutation.</p><p><b>CONCLUSIONS</b>The clinical manifestations were heterogenic within this family. We identified a novel mutation (c.1396delT) was the disease-causing mutation for this family and extended the mutational spectrum of CCMs.</p>

Malformación cavernomatosa: revisión de una patología clásica / Cavernomatous malformations: a classical disease review

Las malformaciones cavernomatosas (MC) son hamartomas vasculares de aspecto mamelonado, inicialmente descritos por Virchow, que son consideradas la malformación vascular más frecuente, después de las anomalías del desarrollo venoso, con una prevalencia de 0,5-0,7 porciento en la población general, sin predilección entre géneros. Se presentan con un amplio comportamiento dinámico en las imágenes dado su diverso grado de crecimiento, la posible regresión, la formación de novo y el riesgo de sangrado. En estas circunstancias, la resonancia magnética (RM), especialmente las potenciaciones en T2 gradiente (T2GRE) y susceptibilidad (SWI), cobran gran importancia en su búsqueda y seguimiento. Se revisa y actualiza su epidemiología y patología; además se presentan sus diversas ubicaciones y características, de tal forma que su diagnóstico no sea complejo.

Cavernomatous malformations (CM) are mamelonated vascular hamartomas, initially described by Virchow, which are considered the most common vascular malformation after developmental venous anomalies, with a prevalence of 0.5-0.7% in the overall population, without gender predilection. In imaging they show a broad range of dynamic behavior given their different levels of growth, possible regression, de novo formation and the risk of bleeding. In these circumstances, magnetic res onance imaging (MRI), especially T2 gradient echo sequence (T2GRE) and susceptibility weighted imaging (SWI), become very important for the detection and follow-up/monitoring of CM.This article reviews and updates the epidemiological and pathological features of CM; as well as presenting its various locations and characteristics, so that the diagnosis is not complicateD.

Giant Cystic Cerebral Cavernous Malformation with Multiple Calcification: Case Report

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2x4.6x6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review.

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.

Is cerebral cavernous malformation a pre-glioma lesion? / 中华医学杂志(英文版)

Glioma is the most malignant tumor in the brain, the origin of glioma is still unknown. Recently some papers indicated that glioma may be developed from cerebral cavernous malformation (CCM). We describe a man with a right temporal lobe CCM, after gamma-knife radiotherapy, the patient developed a low-grade astrocytoma in the area of the preexistent CCM. This case, together with other reports, may indicated an oncogenetic properties of CCM, and we proposed that CCM may be a pre-glioma lesion.

Spinal cavernous malformations: magnetic resonance imaging and associated findings

<p><b>INTRODUCTION</b>We reviewed the clinical features, brain and spinal cord magnetic resonance (MR) imaging findings and associated abnormalities in six patients with spinal cavernous malformations (CMs).</p><p><b>METHODS</b>Lesions were defined on gradient-recalled echo (GRE) images but measured on T2-weighted images performed on 1.5- and 3-tesla clinical scanners.</p><p><b>RESULTS</b>Four patients had associated multiple cranial CMs and one patient had multiple spinal CMs. All spinal CMs were predominantly hypointense on GRE images, and most were predominantly hyperintense and surrounded by hypointense edge on T2-weighted images. Other associations included asymptomatic vertebral body and splenic haemangiomas.</p><p><b>CONCLUSION</b>We conclude that intramedullary spinal CMs typically have 'mulberry' or 'popcorn' appearances similar to those of cranial CM. The presence of associated haemangioma or familial cranial CM syndrome on MR imaging may suggest the correct diagnosis without requiring invasive investigations.</p>

PHACE association with intracranial, oropharyngeal hemangiomas, and an atypical patent ductus arteriosus arising from the tortuous left subclavian artery in a premature infant / 소아과

PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.