[Moya-moya disease in a child with multiple malformations]

Moya-Moya disease is an obstruction of the internal carotids and of the afferent and efferent channels of Willis's polygon, which causes a collateral circulation, responsible for the typical angiographic image of a "puff of smoke" (Moya-Moya, in Japanese). Its etiology is unknown, and it might be congenital or acquired. It has been associated with congenital diseases such as Neurofibromatosis and disease of the falciform cells, among others. It usually appears as an alternating hemiplegia. We report here on the case of a 4-year-old boy with alternating hemiplegia (initially on the left, later on the right and then back on the left) and angiographic alterations which are characteristic of Moya-Moya. This patient had been examined in our hospital in the neonatal period as he had malformative stigmas, basically consistent with a flat angioma on the forehead, upper eyelids, and base of the nose, a previous wide fontanella which expanded to the whole of the methopic suture, aplasia, forehead level skin and abnormal implantation of the skin in that region. What draws attention in this case is the association of middle line craniofacial lesions (angioma, skin aplasia) and the later development of Moya-Moya.