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.

Endovascular Treatment in the Subclavian Steal Syndrome: Series of 29 Patients

Subclavian steal syndrome is a group of symptoms resulting fromretrograde flow in the vertebral artery, "stealing" blood from the posterior intracranial circulation and other territories, caused by stenosis or occlusion of the subclavian artery proximal to the origin of the same vertebral artery, or even of the brachiocephalic trunk. Most of the time, it is an incidental finding in patients with other conditions or cerebrovascular risk factors. We report a series of 29 patients with an angiographic diagnosis, in which 7 received treatment (all endovascular), all with symptoms directly related to this condition. Advanced age, systemic arterial hypertension, diabetes mellitus, smoking and stroke were comorbidities frequently related. Six patients improved completely after the procedure and one remained with vertigo.

Rescue therapy with stent retrievers for thromboembolism during endovascular treatment of intracranial aneurysms / Terapia de resgate com stent para tromboembolismo durante tratamento endovascular de aneurismas intracranianos

ABSTRACT Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneurysm embolization. We retrospectively analyzed 10 consecutive patients with transprocedural arterial occlusion, treated with mechanical thrombectomy at a single center. Good angiographic recanalization was achieved in eight cases, mTICI 3, 2b and 2a in five, three and two patients, respectively, without additional complications or any deaths. Five patients showed complete recovery (mRS 0) and all patients showed improvement of disability (average mRS 1.1) over a mean follow-up period of 31 months. Eight patients had good clinical recovery, while two remained with deficits (mRS 3 and 4). The study found that the stent retriever is a valuable, rapid and effective tool for restoring blood flow, improving the safety of endovascular treatment.

RESUMO Tromboembolismo é a complicação mais frequente no tratamento endovascular de aneurismas cerebrais, podendo causar morte ou sequelas. Como os stent retrievers alcançaram altas taxas de recanalização arterial no tratamento do acidente vascular encefálico isquêmico, testamos esses dispositivos para tratar eventos tromboembólicos ocorridos durante a embolização de aneurismas. Foram analisados retrospectivamente 10 pacientes apresentando oclusão arterial transoperatória, tratados com trombectomia mecânica em um único centro. Obtivemos recanalização angiográfica em oito casos, mTICI 3, 2b e 2a em cinco, três e dois pacientes, respectivamente, sem complicações adicionais ou óbito. Cinco casos apresentaram recuperação completa (mRS 0) e todos os pacientes apresentaram melhora dos déficits (mRS médio 1.1) durante acompanhamento médio de 31 meses. Oito pacientes apresentaram boa recuperação clínica, enquanto dois permaneceram com déficits (mRS 3 e 4). O estudo concluiu que stents são uma ferramenta valiosa, rápida e eficaz para restaurar o fluxo sanguíneo, aumentando a segurança do tratamento endovascular.

Tratamiento endovascular plurimodal de aneurismas múltiples de la circulación anterior. Reporte de un caso / Multimodalities endovascular treatment of multiple aneurysms of the anterior circulation. Case report

Aneurismas cerebrales son una enfermedad grave, a pesar del alto grado de conocimiento respecto su fisiopatología y patogenia. La letalidad de ruptura de un aneurisma cerebral es todavía alrededor de 50%, allá de altas tasas de morbilidad. El tratamiento endovascular ha evolucionado en las últimas décadas para mejorar los resultados. Se presenta el caso de un paciente con síntomas neurológicos graves secundarios a la hemorragia subaracnoidea, cuya investigación mostró múltiples aneurismas. Varios dispositivos fueron utilizados en modalidades terapéuticas endovasculares consecutivas, incluyendo la embolización con remodelación, colocación de stents y desviador de flujo, con buen resultado clínico y angiográfico.

Cerebral aneurysms are a serious illness, despite the high degree of knowledge about its pathophysiology and pathogenesis. The lethality of cerebral aneurysm´s rupture is still about 50% beyond high morbidity. Endovascular treatment has evolved in recent decades to improve outcomes. It is reported the case of a patient with severe neurological symptoms secondary to subarachnoid hemorrhage, whose examination showed multiple aneurysms. Several devices were used on consecutive endovascular therapeutic modalities, including remodeling embolization, stenting and flow diverter, with good clinical and angiographic outcome.