Failure of encephalo-duro-arterio-synangiosis procedure in moyamoya disease.

Moyamoya disease is an uncommon cerebrovascular disease characterized angiographically by progressive stenosis of the distal internal carotid arteries and proximal anterior and middle cerebral arteries coupled with the development of a fine network of vessels at the base of the brain. In children, the disease causes recurrent hemisphere ischemia. A newly described operative procedure (encephalo-duro-arterio-synangiosis) was recently recommended by Matsushima and Inaba [Child's Brain 11: 155-170, 1984] and was used in a recent patient treated at the UCLA Medical Center. The procedure did not succeed in revascularizing the brain in spite of clinical and angiographic progression of the disease, but a conventional superficial temporal to middle cerebral bypass operation did.