Moyamoya disease and syndrome: a review / Doença e síndrome de moyamoya: uma revisão

Abstract Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

RESUMO A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Prenatal Diagnosis of Vein of Galen Aneurysmal Malformation Allows Early Transumbilical Endovascular Treatment

Neonates with vein of Galen aneurysmal malformation (VGAM) presenting with severe cardiac failure and pulmonary hypertension represent a challenge for endovascular therapy.When early treatment is required, the small femoral arteries in this population are usually difficult to cannulate. Alternatively, the umbilical vessels offer a natural pathway to reach the lesion. Therefore, prenatal diagnosis of VGAM allows for delivery planning, perinatal management, and embolization through umbilical approach, thus leading to better outcomes.

Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia

Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with good outcome. All of the cervical and renal arteries were diseased and showed dysplasia and/or ectasias. Conclusions There are no guidelines or protocols to treat patients with FMD.

Henrique Dumont: how a traumatic brain injury contributed to the development of the airplane / Henrique Dumont: como um traumatismo cranioencefálico contribuiu para o desenvolvimento do avião

ABSTRACT The life and death of Henrique Dumont (1832-1892) is little known, being usually remembered as Alberto Santos-Dumont's father. This report describes the history of this Brazilian engineer, also known as the King of Coffee, who achieved enormous business success and fortune in the late nineteenth century. In 1890, during the inspection of his farm, the world's largest coffee plantation at that time, he fell from a carriage, which left him a hemiplegic. This forced him to sell the farm and move to France for treatment. Before his death two years later, he gave his 18-year-old son bits of advice, and distributed his inheritance, which allowed Alberto to study in Paris and finance his experiments that would culminate in the development of the airplane. The diagnostic and therapeutic possibilities are also discussed.

RESUMO A vida e especialmente a morte do brasileiro Henrique Dumont (1832-1892) são pouco conhecidas, sendo geralmente lembrado como pai de Alberto Santos Dumont. Relatamos a história deste engenheiro que obteve enorme sucesso empresarial no final do século XIX, sendo denominado Rei do Café. Em 1890, durante a inspeção da sua fazenda, maior cafeicultura do mundo na época, caiu da charrete e ficou hemiplégico. Isso o fez vender a fazenda e buscar tratamento na França, trazendo o primeiro automóvel do Brasil ao retornar. Antes de morrer dois anos após o acidente, deu valorosos conselhos e distribuiu sua herança ao filho Alberto, então com dezoito anos de idade, que pôde assim estudar em Paris e financiar seus experimentos que culminariam no desenvolvimento do avião. São discutidas e contemporizadas as possibilidades diagnósticas e terapêuticas relacionadas ao déficit neurológico.

Cerebral Proliferative Angiopathy: Case Report

Proliferative angiopathy (PA) is a rare cerebral vascular disease in which anomalous vessels continually recruit additional feeder arteries, amid a functional brain parenchyma. We report the case of a young woman with progressive history of headache, motor deficit, seizures and drowsiness. She received a misdiagnosis of brain arteriovenous malformation (AVM) and evolved with dysarthria and cognitive decline after an unsuccessful embolization performed at another institution. We opted for conservative treatment with periodic control by imaging tests. Proliferative angiopathy differs in natural history, prognosis, histopathology and treatment of the usual AVMs. Endovascular procedures aggravate the neurological deficits, which are usually progressive and tend to worsen over time.

A angiopatia proliferativa (AP) é uma doença vascular cerebral rara em que vasos anômalos recrutam continuamente artérias nutridoras adicionais em um parênquima cerebral normal. Relatamos um caso de uma mulher jovem com história progressiva de cefaleia, déficit motor, convulsões e sonolência. Ela recebeu um diagnóstico incorreto da malformação arteriovenosa (MAV) cerebral e evoluiu com disartria e declínio cognitivo após uma embolização malsucedida realizada em outra instituição. Optamos pelo tratamento conservador com controle periódico por testes de imagem. A AP difere das MAVs usuais em relação a história natural, prognóstico, histopatologia e tratamento. Os procedimentos endovasculares agravam os déficits neurológicos, que geralmente são progressivos e tendem a piorar ao longo do tempo.

Late Coil Migration after Embolization of Cerebral Aneurysms ­ Case Series

Background Coil migration after cerebral aneurysm embolization is a very rare complication. It can take place early or late in the postoperative period, evolving asymptomatic and causing severe neurological deficits. Ruptured aneurysms of anterior communicant artery were the most frequent reported examples in the literature. Case Report We report three cases of patients with unruptured aneurysms treated with coils and remodeling balloon technique. The first case was that of a left posterior communicating artery aneurysm with coil migration to a distal cortical branch of ipsilateral middle cerebral artery (MCA). The patient had mild paresthesia in the arm for a few days. The second one was a fenestrated basilar artery aneurysm with coil migration to the P2 segment of the left posterior cerebral artery. The patient was fully asymptomatic. The third case was a MCA aneurysm with coil migration to the M3 segment. There were no ischemic complications, and all patients underwent a new endovascular procedure. Conclusions Coil migration is a rare but not always severe complication. Antiplatelet agents are recommended even if the coil migration is asymptomatic.

Introdução A migração de coil após embolização de aneurisma cerebral é uma complicação muito rara. Ela pode ocorrer no período pós-operatório tanto precoce quanto tardio, evoluindo assintomático ou causando graves déficits neurológicos. Aneurismas rotos da artéria comunicador anterior foram os exemplos mais frequentes relatados na literatura. Relato de Caso Relatamos três casos de pacientes com aneurismas não rotos tratados com coils e técnica de remodelagem com balão. O primeiro caso foi de um aneurisma de artéria comunicante posterior com migração do coil para um ramo cortical distal da artéria cerebral média (ACM) ipsilateral. A paciente apresentou parestesia leve no braço por alguns dias. O segundo foi um aneurisma de artéria basilar fenestrada com migração de coil para o segmento P2 da artéria cerebral posterior esquerda. O paciente ficou totalmente assintomático. O terceiro caso foi de um aneurisma de ACM com migração do coil para o segmento M3. Não houve complicações isquêmicas, e todos os pacientes foram submetidos a novo procedimento endovascular. Conclusões Migração de coil é uma complicação rara, mas nem sempre grave. Antiagregantes plaquetários são recomendados mesmo se a migração do coil for assintomática.

Endovascular Treatment of a Giant Dissecting Aneurysm in an Infant

Introduction The incidence of intracranial aneurysms in the pediatric population is low, and endovascular treatment is becoming a safe and minimally invasive treatment option. In the present study, the occurrence of special features of cerebral aneurysm in children, in comparison to adults, is also described. Case Report A 3-month-old female infant presented with progressive proptosis and divergent strabismus at the right eye, in addition to inconsolable crying. Cerebral resonance, angiotomography and angiography exams demonstrated angiodysplasia in the right internal carotid artery with two large paraclinoid dissecting aneurysms with wide neck. The right internal carotid artery was occluded with coils by endovascular approach, without detriment to the perfusion of the ipsilateral hemisphere and without neurological deficits. The patient achieved good recovery, and a late control angiotomography confirmed the exclusion of the aneurysms. Conclusion Parent artery sacrifice via endovascular approach is an effective therapeutic option, but a long-termfollow-up is necessary to avoid recurrence and bleeding.

Postoperative Dural Arteriovenous Fistula after Clipping of Ruptured Saccular Aneurysm of Pericallosal Artery

Dural arteriovenous fistulas (DAVFs) are pathologic dural shunts characterized by meningeal arterial supply, absence of nidus and drainage to dural venous sinuses or cortical veins. They are usually acquired lesions associated with specific disease and inflammatory processes, including craniotomy. We report a case of postoperative DAVF in a 59 year-old male presenting with a ruptured distal right anterior cerebral artery aneurysm. After an uneventful microsurgery for clipping, meningitis occurred on the ninth day, and was successfully treated. The one-year control angiogram showed a left occipital DAVF. The relevant literature on the occurrence of DAVFs is also presented.

Fístulas arteriovenosas durais (FAVDs) são comunicações patológicas caracterizadas por suprimento arterial meníngeo, ausência de nidus e drenagem para seios venosos durais ou veias corticais. As fístulas são geralmente adquiridas, secundárias a processos inflamatórios e doenças específicas, podendo ocorrer após craniotomia. Relatamos um caso de FAVD ocorrido em um homem de 59 anos de idade com aneurisma intracraniano roto em artéria cerebral anterior direita distal, submetido a microcirurgia e clipagem, e evoluindo com meningite pós-operatória. O paciente apresentou boa evolução, mas na angiografia de controle um ano após a cirurgia foi identificada FAVD em seio transverso-sigmoide esquerdo. Uma revisão da literatura focada na etiologia também é apresentada.