[Moyamoya disease: advantage of early diagnosis and survival treatment. Review of three cases]. / Maladie de Moya-Moya: intérêt d'un diagnostic et d'un traitement chirurgical précoces. A propos de trois observations.

A diagnosis of moyamoya disease was made in three children aged five, eight and 13 years (including two Turkish sisters). Clinical presentation was recurrent episodes of cerebral ischemia and stroke. CT scans and MRI showed infarcts in various distributions. Angiography revealed anterior bilateral stenosis of the circle of Willis and development of Moyamoya collateral pathways. In one case there was coagulopathy with protein C deficiency. To increase transdural collateral flow, revascularisation with encephalo-duro-arterio-synangiosis was attempted in all three children. Outcome was clinically and angiographically satisfactory and none of the children developed further neurological complications. The current state of study on Moyamoya disease is also presented.