The current clinical picture of cerebral proliferative angiopathy: systematic review.

BACKGROUND: Cerebral proliferative angiopathy (CPA) is a rare subset of arteriovenous malformations (AVM). It has unique clinical presentation, angiographic characteristics, and pathophysiology which often brings challenges for the treatment. We aimed to define its epidemiology, pathophysiology are unknown, and best management strategies. METHODS: A systematic review was conducted according to the PRISMA guidelines. MEDLINE was searched for articles regarding CPA. Extracted data included epidemiological, clinical, and angiographical characteristics, treatment, and outcomes. Treatment was classified as conservative, radiosurgery, endovascular, decompression, and indirect vascularization. A meta-analytical approach was employed for description of the data as study-size adjusted percentages or weighted means, as appropriate. RESULTS: Thirty-three studies were analyzed, rendering a total 95 cases-half of which came from a single study. Patients were predominantly young (mean 23 years old) and female (60.0%) presenting with headaches (44.9%), seizures (37.1%), or transient ischemic attacks (33.7%). Hemorrhage was present in 18.0%, but rebleeding rates were as high as 67%. The majority of nidus were > 6 cm (52.5%) with hemispheric extension (73.0%). Capillary angioectatic appearance (85.7%), transdural supply (62.5%), and deep venous drainage (73.0%) were also frequent features. Most patients were treated conservatively (54.4%), followed by endovascular (34.2%). Indirect vascularization and radiosurgery were attempted in five and two patients, respectively. Mean follow-up was 110.8 patient-years. Neurological status improved in 50.7%, remained stable in 40.2%, and worsened in 9.0%. CONCLUSIONS: Conservative and endovascular treatments seem adequate interventions, despite limited evidence. Complementary techniques can be used in patients throughout disease history, according to symptom-based, individualized approach. More studies are required for choosing interventions based on reliable long-term results.

Post-traumatic carotid-cavernous fistula in a pediatric patient: a case-based literature review.

BACKGROUND: Carotid-cavernous fistula (CCF) is a shunt between the carotid artery and the cavernous sinus. Traumatic CCFs are diagnosed in 0.2% of head traumas being only 4.6% of the pediatric population. Classified by Barrow in 1985, type A CCF is the most frequent, occurring in 75% of cases. Type A is characterized by direct and high-flow CCF that generally can occur as a result of traumatic injury or rupture of an intracavernous aneurysm. CASE PRESENTATION: The subject was an 8-year-old boy with penetrating trauma to his left eye. During the initial evaluation, a computed tomography (CT) scan was unremarkable, and after relief of symptoms, the patient was discharged. Seven days later, he developed grade I proptosis, conjunctival chemosis, ophthalmoplegia (III, IV, and VI cranial nerve palsies), and left-sided ptosis and mydriasis. Arteriography confirmed a post-traumatic CCF, and the patient was treated with an endovascular detachable balloon. CONCLUSION: CCF should be suspected in craniofacial traumas with ocular symptoms. The presence of a skull base fracture on CT is a poor predictor of CCF associated with head trauma. Early diagnosis and treatment can prevent permanent neurological deficits and unfavorable outcomes.

Acute and chronic changes in porcine rete mirabile after embolization with the Menox system: angiographic and histopathological analyses.

Objective: To evaluate acute and chronic changes seen on angiographic and histopathological studies of porcine rete mirabile, comparing those treated with the Menox liquid embolic system (LES) and those treated with the Onyx LES. Materials and Methods: Five pigs, each weighing approximately 35 kg, were submitted to rete mirabile embolization under general anesthesia and fluoroscopic guidance, with the Menox LES or Onyx LES. Four animals were treated with the Menox LES and underwent cerebral angiography, followed by euthanasia, at 1, 30, 60, and 90 days after embolization. One animal was treated with the Onyx LES underwent the same procedures at 30 days after embolization. In a subsequent histopathological analysis, we compared the Menox LES and Onyx LES in terms of the acute and chronic changes observed. Results: We observed no significant changes in blood pressure, heart rate, or electrocardiographic parameters that could be attributed to the super-selective infusion of dimethyl sulfoxide or the Menox embolic agent. Fluoroscopy showed adequate material opacity, appropriate progression to the center of the rete mirabile and complete unilateral embolization. Microcatheters were uneventfully detached from the embolized nidus. We observed mild to moderate intravascular and extravascular inflammatory responses, without histological evidence of necrotizing arteritis. There were no adverse neurovascular events. Conclusion: The Menox LES appears to be safe and effective, as well as being apparently equivalent to the Onyx LES in terms of the postprocedure angiographic and histopathological findings.

Objetivo: Avaliar as alterações angiográficas e histopatológicas agudas e crônicas em rete mirabile suína tratadas com o Menox liquid embolic system (LES) e comparar essas alterações com a embolização com Onyx LES. Materiais e Métodos: A embolização da rete mirabile com Menox LES e Onyx LES foi realizada em cinco suínos pesando cerca de 35 kg sob anestesia geral e orientação fluoroscópica. Quatro animais tratados com Menox LES foram submetidos a angiografia cerebral seguida de eutanásia após 1, 30, 60 e 90 dias e um animal tratado com Onix LES foi submetido ao mesmo procedimento após 30 dias. A análise histopatológica subsequente para alterações agudas e crônicas avaliou o desempenho do Menox LES comparado ao Onyx LES. Resultados: Não foram observadas alterações significativas atribuíveis à infusão superseletiva de dimetilsulfóxido ou Menox nos parâmetros de pressão arterial, frequência cardíaca ou eletrocardiograma. A fluoroscopia mostrou opacidade adequada do material, progressão adequada para o centro da rete mirabile e embolização unilateral completa. Os microcateteres foram retirados do nidus embolizado sem complicações. Observou-se resposta inflamatória intravascular e extravascular leve a moderada, sem indício histológico de arterite necrosante. Nenhum dos casos apresentou eventos neurovasculares adversos. Conclusão: A injeção de Menox LES mostrou-se segura e eficaz, além de ser equivalente ao Onyx LES em relação aos achados angiográficos e histopatológicos pós-procedimento.

Perimedullary arteriovenous fistulas in children: report on six cases.

BACKGROUND: Perimedullary arteriovenous fistulas (PMAVFs) are rare spinal lesions and even more uncommon in children. OBJECTIVE: The aim of this study was to document rare occurrences of this type of arteriovenous malformation in six children treated at our institution. METHODS: The clinical data, radiological findings, and treatment in six cases of PMAVFs were reviewed. Six patients with PMAVFs were managed at our institution over a 5-year period. The patients (four girls and two boys), ranging in age from 6 to 15 years, presented with initially fluctuating, and eventually permanent and progressive, sudden-onset paraparesis, sensory disturbances, and sphincter dysfunction. The duration of symptoms before diagnosis ranged from 1 week to 13 years. RESULTS: All the patients underwent magnetic resonance imaging and spinal selective angiography, which demonstrated the characteristic imaging of an arteriovenous fistula. Embolization of the arteriovenous fistula was initially attempted in three patients with successful occlusion of the fistula in two. For the remaining cases, open surgery was performed, with complete occlusion of the fistula. There was no morbidity, regardless of the treatment performed. All the patients experienced neurological improvement after treatment. CONCLUSIONS: No specific clinical or radiological characteristic of PMAVFs in the pediatric population was observed when our series was compared with a general series. Early diagnosis and timing of the therapeutic intervention seemed to avoid the development of irreversible ischemic myeloradiculopathy and prevented hemorrhage. Treatment for PMAVFs is difficult to standardize because these are extremely rare lesions with different angioarchitecture configurations.

Ethanol sclerotherapy of superficial venous malformation: a new procedure.

BACKGROUND: Superficial venous malformations (SVM) are the most frequent vascular malformations. Outpatient percutaneous treatment with ethanol injection has rarely been described. OBJECTIVE: To analyze the results from treating SVM patients with ethanol sclerotherapy. METHODS: 81 patients were followed up prospectively over a median period of 18 months. 47 were female and 34 were male with a median age of 21 years. Most of the lesions were located in the limbs and the main symptom reported was pain. Each patient underwent fortnightly alcohol injection sessions. RESULTS: The lesions and the symptoms completely disappeared or improved in 77 patients. There were no complications in 64 patients, while 11 presented a small skin ulcer, 3 hyperpigmentation and 3 paresthesia. CONCLUSION: Ethanol sclerotherapy for SVM patients was effective, with a low complication rate.

Management of acute stroke and urgent neurointerventional procedures during COVID-19 pandemic: recommendations on the Scientific Department on Cerebrovascular Diseases of the Brazilian Academy of Neurology, Brazilian Society of Cerebrovascular Diseases and Brazilian Society of Neuroradiology.

INTRODUCTION: Although the 2019 severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2, COVID-19) pandemic poses new challenges to the healthcare system to provide support for thousands of patients, there is special concern about common medical emergencies, such as stroke, that will continue to occur and will require adequate treatment. The allocation of both material and human resources to fight the pandemic cannot overshadow the care for acute stroke, a time-sensitive emergency that with an inefficient treatment will further increase mortality and long-term disability. OBJECTIVE: This paper summarizes the recommendations from the Scientific Department on Cerebrovascular Diseases of the Brazilian Academy of Neurology, the Brazilian Society of Cerebrovascular Diseases and the Brazilian Society of Neuroradiology for management of acute stroke and urgent neuro-interventional procedures during the COVID-19 pandemic, including proper use of screening tools, personal protective equipment (for patients and health professionals), and patient allocation.

Acute and chronic changes in porcine rete mirabile after embolization with the Menox system: angiographic and histopathological analyses / Alterações angiográficas e histopatológicas agudas e crônicas da rete mirabile no modelo suíno com sistema embólico líquido Menox

Abstract Objective: To evaluate acute and chronic changes seen on angiographic and histopathological studies of porcine rete mirabile, comparing those treated with the Menox liquid embolic system (LES) and those treated with the Onyx LES. Materials and Methods: Five pigs, each weighing approximately 35 kg, were submitted to rete mirabile embolization under general anesthesia and fluoroscopic guidance, with the Menox LES or Onyx LES. Four animals were treated with the Menox LES and underwent cerebral angiography, followed by euthanasia, at 1, 30, 60, and 90 days after embolization. One animal was treated with the Onyx LES underwent the same procedures at 30 days after embolization. In a subsequent histopathological analysis, we compared the Menox LES and Onyx LES in terms of the acute and chronic changes observed. Results: We observed no significant changes in blood pressure, heart rate, or electrocardiographic parameters that could be attributed to the super-selective infusion of dimethyl sulfoxide or the Menox embolic agent. Fluoroscopy showed adequate material opacity, appropriate progression to the center of the rete mirabile and complete unilateral embolization. Microcatheters were uneventfully detached from the embolized nidus. We observed mild to moderate intravascular and extravascular inflammatory responses, without histological evidence of necrotizing arteritis. There were no adverse neurovascular events. Conclusion: The Menox LES appears to be safe and effective, as well as being apparently equivalent to the Onyx LES in terms of the postprocedure angiographic and histopathological findings.

Resumo Objetivo: Avaliar as alterações angiográficas e histopatológicas agudas e crônicas em rete mirabile suína tratadas com o Menox liquid embolic system (LES) e comparar essas alterações com a embolização com Onyx LES. Materiais e Métodos: A embolização da rete mirabile com Menox LES e Onyx LES foi realizada em cinco suínos pesando cerca de 35 kg sob anestesia geral e orientação fluoroscópica. Quatro animais tratados com Menox LES foram submetidos a angiografia cerebral seguida de eutanásia após 1, 30, 60 e 90 dias e um animal tratado com Onix LES foi submetido ao mesmo procedimento após 30 dias. A análise histopatológica subsequente para alterações agudas e crônicas avaliou o desempenho do Menox LES comparado ao Onyx LES. Resultados: Não foram observadas alterações significativas atribuíveis à infusão superseletiva de dimetilsulfóxido ou Menox nos parâmetros de pressão arterial, frequência cardíaca ou eletrocardiograma. A fluoroscopia mostrou opacidade adequada do material, progressão adequada para o centro da rete mirabile e embolização unilateral completa. Os microcateteres foram retirados do nidus embolizado sem complicações. Observou-se resposta inflamatória intravascular e extravascular leve a moderada, sem indício histológico de arterite necrosante. Nenhum dos casos apresentou eventos neurovasculares adversos. Conclusão: A injeção de Menox LES mostrou-se segura e eficaz, além de ser equivalente ao Onyx LES em relação aos achados angiográficos e histopatológicos pós-procedimento.

Do leptomeningeal venous drainage and dysplastic venous dilation predict hemorrhage in dural arteriovenous fistula?

BACKGROUND: The objective of this study was to correlate the presence of leptomeningeal venous drainage and dysplastic venous dilation with the risk of intracranial hemorrhage in DAVFs. METHODS: The subjects for this research were composed of 93 patients with DAVFs who were studied retrospectively with regard to therapeutic success and failure, who had undergone either neurosurgery or embolization or a combination of both methods, and whose disease was located in the cavernous sinus, the superior sagittal sinus, or the transverse-sigmoid sinus of the anterior fossa or of the tentorium. Also among these study subjects were patients who had had angiography done in at least 6 cranial vessels (external and internal carotid and vertebral arteries) before and after treatment and who had a minimum follow-up of 3 months. Cases of leptomeningeal venous drainage and dysplastic venous dilation and their associations with intracranial hemorrhage were ascertained for all the 93 case patients included in the study. RESULTS: The presence of leptomeningeal drainage (P = .0002) and that of dysplastic venous dilation (P = .036) increased the risk of intracranial hemorrhage in case patients for DAVFs of the 5 regions. CONCLUSION: There is a statistical significance between risk of intracranial hemorrhage in DAVFs and the presence of leptomeningeal drainage and dysplastic venous dilation.

Analysis of the best therapeutic alternative for intracranial dural arteriovenous malformations.

The material for this research consisted of 93 patients with dural arteriovenous malformations (DAVMs) who were studied retrospectively with regards to therapeutic success and failure, who had undergone either neurosurgery, or embolization or a combination of both methods and whose disease was located in the cavernous sinus, the superior sagittal sinus, the transverse-sigmoid sinus of the anterior fossa and the tentorium. Thus, it was possible to arrive at the following conclusions: treatment of the DAVMs must be indicated, jointly, by an interventionist neuroradiologist and a neurosurgeon; DAVMs of the transverse-sigmoid sinus were better treated when a combination of both methods was used; DAVMs of the tentorium were also better treated with a combined method; the endovascular method ensured only a 50% chance of therapeutic success for DAVMs of the superior sagittal sinus; DAVMs of the cavernous sinus are better treated when the endovascular method was used with a transvenous approach, relative to the transarterial approach.

Image-Guided Cryoablation of the Spine in a Swine Model: Clinical, Radiological, and Pathological Findings with Light and Electron Microscopy.

PURPOSE: This study was designed to present the feasibility of an in vivo image-guided percutaneous cryoablation of the porcine vertebral body. METHODS: The institutional animal care committee approved this study. Cone-beam computed tomography (CBCT)-guided vertebral cryoablations (n = 22) were performed in eight pigs with short, 2-min, single or double-freezing protocols. Protective measures to nerves included dioxide carbon (CO2) epidural injections and spinal canal temperature monitoring. Clinical, radiological, and pathological data with light (n = 20) or transmission electron (n = 2) microscopic analyses were evaluated after 6 days of clinical follow-up and euthanasia. RESULTS: CBCT/fluoroscopic-guided transpedicular vertebral body cryoprobe positioning and CO2 epidural injection were successful in all procedures. No major complications were observed in seven animals (87.5 %, n = 8). A minor complication was observed in one pig (12.5 %, n = 1). Logistic regression model analysis showed the cryoprobe-spinal canal (Cp-Sc) distance as the most efficient parameter to categorize spinal canal temperatures lower than 19 °C (p < 0.004), with a significant Pearson's correlation test (p < 0.041) between the Cp-Sc distance and the lowest spinal canal temperatures. Ablation zones encompassed pedicles and the posterior wall of the vertebral bodies with an inflammatory rim, although no inflammatory infiltrate was depicted in the surrounding neural structures at light microscopy. Ultrastructural analyses evidenced myelin sheath disruption in some large nerve fibers, although neurological deficits were not observed. CONCLUSIONS: CBCT-guided vertebral cryoablation of the porcine spine is feasible under a combination of a short freezing protocol and protective measures to the surrounding nerves. Ultrastructural analyses may be helpful assess the early modifications of the nerve fibers.

Validation of cone-beam computed tomography and magnetic resonance imaging of the porcine spine: a comparative study with multidetector computed tomography and anatomical specimens.

BACKGROUND CONTEXT: New spinal interventions or implants have been tested on ex vivo or in vivo porcine spines, as they are readily available and have been accepted as a comparable model to human cadaver spines. Imaging-guided interventional procedures of the spine are mostly based on fluoroscopy or, still, on multidetector computed tomography (MDCT). Cone-beam computed tomography (CBCT) and magnetic resonance imaging (MRI) are also available methods to guide interventional procedures. Although some MDCT data from porcine spines are available in the literature, validation of the measurements on CBCT and MRI is lacking. PURPOSE: To describe and compare the anatomical measurements accomplished with MDCT, CBCT, and MRI of lumbar porcine spines to determine if CBCT and MRI are also useful methods for experimental studies. STUDY DESIGN: An experimental descriptive-comparative study. METHODS: Sixteen anatomical measurements of an individual vertebra from six lumbar porcine spines (n=36 vertebrae) were compared with their MDCT, CBCT, and MRI equivalents. Comparisons were made for the absolute values of the parameters. RESULTS: Similarities were found in all imaging methods. Significant correlation (p<.05) was observed with all variables except those that included cartilaginous tissue from the end plates when the anatomical study was compared with the imaging methods. CONCLUSIONS: The CBCT and MRI provided imaging measurements of the lumbar porcine spines that were similar to the anatomical and MDCT data, and they can be useful for specific experimental research studies.

Management of acute stroke and urgent neurointerventional procedures during COVID-19 pandemic: recommendations on the Scientific Department on Cerebrovascular Diseases of the Brazilian Academy of Neurology, Brazilian Society of Cerebrovascular Diseases and Brazilian Society of Neuroradiology / Tratamento do acidente vascular cerebral agudo e realização de procedimentos de neurointervenção urgentes durante a pandemia de COVID-19: recomendações do Departamento Científico de Doenças Cerebrovasculares da Academia Brasileira de Neurologia, Sociedade Brasileira de Doenças Cerebrovasculares e Sociedade Brasileira de Neurorradiologia

ABSTRACT Introduction: Although the 2019 severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2, COVID-19) pandemic poses new challenges to the healthcare system to provide support for thousands of patients, there is special concern about common medical emergencies, such as stroke, that will continue to occur and will require adequate treatment. The allocation of both material and human resources to fight the pandemic cannot overshadow the care for acute stroke, a time-sensitive emergency that with an inefficient treatment will further increase mortality and long-term disability. Objective: This paper summarizes the recommendations from the Scientific Department on Cerebrovascular Diseases of the Brazilian Academy of Neurology, the Brazilian Society of Cerebrovascular Diseases and the Brazilian Society of Neuroradiology for management of acute stroke and urgent neuro-interventional procedures during the COVID-19 pandemic, including proper use of screening tools, personal protective equipment (for patients and health professionals), and patient allocation.

RESUMO Introdução: A pandemia causada pelo novo coronavírus da síndrome respiratória aguda grave 2 (SARS-CoV-2, COVID-19) apresenta novos e importantes desafios à gestão de saúde no Brasil. Além da difícil missão de prestar atendimento aos milhares de pacientes infectados pelo COVID-19, os sistemas de saúde têm que manter a assistência às emergências médicas comuns em períodos sem pandemia, tais como o acidente vascular cerebral (AVC), que continuam ocorrendo e requerem tratamento com presteza e eficiência. A alocação de recursos materiais e humanos para o enfrentamento à pandemia não pode comprometer o atendimento ao AVC agudo, uma emergência cujo tratamento é tempo-dependente e se não realizado implica em importante impacto na mortalidade e incapacitação a longo prazo. Objetivo: Este trabalho resume as recomendações do Departamento Científico de Doenças Cerebrovasculares da Academia Brasileira de Neurologia, da Sociedade Brasileira de Doenças Cerebrovasculares e da Sociedade Brasileira de Neurorradiologia para o tratamento do AVC agudo e para a realização de procedimentos de neurointervenção urgentes durante a pandemia de COVID-19, incluindo o uso adequado de ferramentas de triagem e equipamentos de proteção pessoal (para pacientes e profissionais de saúde), além da alocação apropriada de pacientes.

Treatment of subclavian steal syndrome with percutaneous transluminal angioplasty and stenting: case report.

Subclavian steal syndrome refers to the association of neurological symptoms related to vertebrobasilar insufficiency and the phenomenon of subclavian steal. We report the case of a 63 year-old male patient that presented subclavian steal syndrome and severe proximal (80%) stenosis of the left subclavian artery. The patient was submitted to percutaneous transluminal angioplasty and stenting on the left SA. The procedure was well tolerated and immediately afterwards, there was complete remission of the symptoms and of the phenomenon of subclavian steal evaluated by angiography and transcranial doppler. We propose that percutaneous transluminal angioplasty with stenting placement is a good therapeutic option for subclavian steal syndrome.

Ethanol sclerotherapy of head and neck venous malformations.

OBJECTIVE: This retrospective study evaluated the results of sclerotherapy with low doses of ethanol for treatment of head and neck venous malformations. METHODS: We treated 51 patients, 37 females. Median age was 23 years. Patients were treated with percutaneous intralesional injection of alcohol every two weeks and followed up prospectively for a median period of 18 months. Most lesions affected the face and cosmetic disfigurement was the most frequent complaint. RESULTS: We performed a median of 7 sessions of sclerotherapy. Complete resolution or improvement was observed in 48 patients presented. Five cases of small skin ulceration, two cases of hyperpigmentation and two of paresthesia were documented; all of them were treated conservatively. CONCLUSION: Percutaneous sclerotherapy with low doses of ethanol is a safe and effective treatment modality for venous malformations affecting the head and neck.

Low-grade Endometrial Stromal Sarcoma with Unusual Metastasis to the Skull

Background Endometrial stromal sarcomas (ESSs) are the second most common uterine sarcomas. Although ESSs are often indolent, they have metastatic potential. To the best of our knowledge, there are only three reports of brain metastasis, and the present report is the first to describe a late skull metastasis of an ESS. Case Report We describe the case of a 51-year-old woman who presented abnormal vaginal bleeding 14 years ago; she was diagnosed with an uterine mass and submitted to a hysterectomy. One year ago she presented ESS lung metastasis followed by a left parietal calvarial metastasis. The optimal treatment for metastatic ESS is controversial, but the use of progesterone and aromatase inhibitors is advisable.

Introdução Sarcoma endometrial estromal (SEE) é a segunda lesão mais frequente dentre os sarcomas uterinos. Geralmente são lesões indolentes, mas com potencial de desenvolver metástase. Até o momento há apenas três relatos de metástase cerebral, sendo este o primeiro estudo a descrever uma metástase craniana tardia dessas lesões. Relato de caso Nós descrevemos o caso de uma paciente de 51 anos de idade que apresentou há 14 anos um quadro de sangramento vaginal anormal, sendo diagnosticada uma massa uterina; a paciente foi submetida a uma histerectomia. Há um ano ela evoluiu com metástase pulmonar, seguida por metástase craniana parietal esquerda. O tratamento ideal do SEE metastático ainda é controverso, mas o uso de inibidores de aromatase é aconselhável.