[Involvement of the large vessels supplying the brain in sickle cell disease: a possible cause of Moya-moya (author's transl)].

In two children (1 girl and 1 boy aged 6 years) with an SS haemoglobinopathy, a neurological deficit affecting one half of the body led to arteriograms being performed. These revealed stenoses of the major vessels (above all anterior and middle cerebral with development of a collateral circulation giving an appearance suggestive of Moya-moya syndrome). Various aetiologies have been suggested for this syndrome: congenital disease, oral contraceptives, atheroma. Sickle cell disease is a new cause. The mechanism would appear to be fibrosis proliferating secondary to sub-endothelial anoxia caused by sludging in the vasa vasorum.