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Ventral porencephaly: A cerebral defect associated with multiple congenital anomalies
Authors:R Malcolm Stewart  Roger S Williams  Petr Lukl  Jean Schoenen
Institution:(1) Departments of Neurology and Neuropathology, Massachusetts General Hospital, 200 Trapelo Road, 02154 Waltham, MA, USA;(2) the Eunice Kennedy Shriver Center for Mental Retardation, Inc., Boston, MA, USA;(3) Department of Pathology, Cambridge Hospital, Harvard Medical School, Boston, MA, USA
Abstract:Summary An infant with multiple congenital anomalies was found at autopsy to have a porencencephalic defect on the ventral surface of the left frontal lobe. The intracranial defect was seen in association with an anomalous configuration of the circle of Willis. The zone of tissue destruction corresponded to the vascular territory of the anterior choroidal and lenticulo-striate branches of the proximal middle cerebral arteries, which were absent on the left. The developmental anomaly of the circle of Willis may have predisposed to tissue destruction by compromising cerebral perfusion at midgestation, a stage of rapid brain growth.Supported in part by USPS grants. M 16674, MH 25515 and MH 01215
Keywords:Porencephaly  Perfusion failure  Circle Willis
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