La maladie de Moya-Moya est une artériopathie chronique pouvant toucher l’enfant comme l’adulte, et donnant lieu à des manifestations cliniques sévères d’origine ischémique ou hémorragique. L’imagerie a un rôle central dans le diagnostic, la stratégie thérapeutique et le suivi de la maladie. L’objectif de cette revue de la littérature est de dégager l’utilité, les avantages et les limites de chaque modalité d’imagerie dans cette pathologie. L’angiographie par cathéter et les techniques de médecine nucléaire restent les examens de référence pour l’évaluation morphologique et hémodynamique. Néanmoins, l’IRM, non invasive et non irradiante occupe une place croissante dans la prise en charge.

Moya-Moya disease is a rare arterial occlusive disease affecting the internal carotid artery and its branches. It is found in both pediatric and adult populations, and it may lead to severe clinical presentations such as stroke and intracranial hemorrhage. Several surgical procedures have been developed to improve its clinical outcome. Imaging techniques have a key role in management of Moya-Moya disease, as they are necessary for diagnosis, choice of treatment and follow-up. Although catheter angiography remains the diagnostic gold standard, and nuclear-medicine techniques best perform hemodynamic studies, less invasive imaging techniques have become efficient in serving these purposes. Conventional MRI and MR angiography, as well as MR functional and metabolic studies, are now widely used in each stage of disease management, from diagnosis to follow-up. CT scan and Doppler sonography may also help assess severity of disease and effects of treatment. The aim of this review is to clarify the utility, efficiency and latest developments of each imaging modality in management of Moya-Moya disease.