Abstract
Recurring cerebrovascular disease is extremely rare in children. Moya-moya (supraclinoid carotid stenosis with multiple cerebral telangiectasias) may be the vascular pathology underlying many such instances. Initially described as a congenital anomaly in the Japanese literature, it is uncommonly recognized in occidentals. We have cared for two Caucasian children in whom several factors strongly suggest moya-moya can be an acquired process and in whom the course was much more benign that commonly described. A 4½ year old boy with transposition of the great vessels developed a right hemiparesis after a total corrective operation. Six and one-half years later he had a subarachnoid hemorrhage (SAH). Computed tomography and cerebral angiography demonstrated a left frontal intracerebral hematoma and subadjacent brain atrophy and moya-moya. He has been well for 2½ years. A girl with sickle cell disease had a right hemiparesis at age 8. Six years later she had a SAH. Computed tomography and cerebral angiography demonstrated left parietal atrophy, intracerebral hematoma, and moya-moya. She has been normal for 2 years. Presentation of moya-moya in children as SAH has been noted only once previously. Moreover, our two children had a basic disease process not associated with cerebral telangiectasia but occasionally associated with cerebrovascular occlusive disease. The demonstration of moya-moya six years after a single presumably vascular occlusive event strongly suggests moya-moya can be a tenuous physiologic compensation to an acquired phenomenon.
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Crosley, C. 1120 MOYA-MOYA IN AMERICAN CHILDREN. Pediatr Res 12 (Suppl 4), 550 (1978). https://doi.org/10.1203/00006450-197804001-01126
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DOI: https://doi.org/10.1203/00006450-197804001-01126
