Congenital Porencephaly

Abstract

Congenital porencephaly originates early in fet al development as a component of diffuse cerebral malformation. In infancy the patients have clinical manifestations of local or generalized cerebral disease. Convulsions, delaved growth and development and focal motor deficits are the most common clinical findings. Skull films, transillumination and brain scanning are useful methods of examination. Intracranial pneumography is the definitive means of diagbnosis. Roentgen studies in unilateral cases demonstrate characteristic changes. This pattern consists of ipsilateral calvarial enlargement and thinning, bilateral ventricular dilatation and contralateral shift of midline structures. Other anomalies of the cerebrum and meninges are often demonstrated on roentgen studies. The prognosis in these patients is guarded. Significant mental and motor retardation occurred in nearly all cases.