Enfermedad moyamoya: presentación de un caso y revisión de la literatura / Moyamoya disease: case report and literature review

La Enfermedad Moyamoya es una arteriopatia oclusiva progresiva de los vasos cerebrales, específicamente de la porción distal de las arterias carótidas internas, por lo general con compromiso bilateral. Esta oclusión trae como consecuencia dilatación de los vasos sanguíneos colaterales con el objetivo de convertirse en vías de circulación colateral, de donde la enfermedad adopta su nombre, debido a la apariencia angiográfica de “nube de humo” de estos pequeños vasos dilatados. Clínicamente se manifiesta como eventos cerebrovasculares isquémicos debido a la oclusión de los vasos mencionados o como eventos hemorrágicos debido a la ruptura de los pequeños vasos sanguíneos dilatados. Presentamos el caso de una paciente femenina de 38 años, que acude al hospital en estado de coma, cuya tomografía computarizada revela hemorragia intraventricular que fue tratada con derivación ventricular externa más activador de plasminógeno tisular. Posterior a su estabilización clínica se realizó angiografía cerebral diagnóstica con hallazgos compatibles con Enfermedad Moyamoya. Se decidió realizar subduro-sinangiosis con el fin de inducir la formación de circulación colateral cerebral. Debido a la baja incidencia de la Enfermedad Moyamoya en Latinoamérica, generalmente no es incluida en el diagnóstico diferencial de los eventos cerebrales vasculares, por lo que recomendamos su sospecha en pacientes jóvenes y sin factores de riesgo que debutan con este tipo de eventos cerebrales.

Moyamoya disease is a progressive occlusive disease of the cerebral vessels, specifically the distal portion of the internal carotid arteries, usually with bilateral involvement. This occlusion results in dilatation of collateral blood vessels in order to form collateral circulation pathways, from which the disease takes its name due to the angiographic appearance of “puff of smoke” of these small dilated vessels. Clinically it manifests as ischemic cerebrovascular events due to occlusion of the vessel mentioned or hemorrhagic events due to rupture of small dilated blood vessels. We report the case of a female patient of 38 years old who was admitted to the hospital in coma state, the CT scan revealed intraventricular hemorrhage that was treated with an external ventricular derivation and tissue plasminogen activator. After clinical stabilization diagnostic cerebral angiography was performed with findings consistent with Moyamoya disease. We decided to perform a subduro-sinangiosis, in order to induce the formation of cerebral collateral circulation. Due to the low incidence of Moyamoya disease in Latin America, generally it is not included in the differential diagnosis of cerebral vascular events, we recommend suspect it in young patients without risk factors who present with this type of brain events.

Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III / Moyamoya disease in a girl with Down syndrome. Report of one case

Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the ¡eft middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability.