ABSTRACT
The authors report two cases of Adams-Oliver syndrome in 2-year-old children characterized by aplasia cutis congenita and terminal congenital abnormalities of the limbs. The diagnosis was made at birth and the aplasia cutis was associated with extensive skull defects, exposing the dural sinuses. The differences between the two patients were essentially the extension and the severity of the scalp and limb osteo-cutaneous lesions, associated malformations of the central nervous system and complications. In one child we found focal hemimegalencephaly of the right hemisphere and in the other one the syndrome was complicated by encephaloclastic cerebral lesions and encephalic herniation. Both children have survived, but the diagnosis of central nervous system malformations and the encephaloclastic lesions associated modified the initial prognosis and the future outcome conspicuously.
