Prolonged survival with hydranencephaly: Report of two patients and literature review

Infants with hydranencephaly are presumed to have a reduced life expectancy , with a survival of several weeks to months. Rarely, patients with prolong ed survival have been reported, but these infants may have had other neurol ogic conditions that mimicked hydranencephaly, such as massive hydrocephalu s or holoprosencephaly, We report two infants with prenatally acquired hydr anencephaly who survived for 66 and 24 months. We reviewed published report s to ascertain the clinical and laboratory features associated with surviva l of more than 6 months. This review demonstrates that prolonged survival u p to 19 years can occur with hydranencephaly, even without rostral brain re gions, with isoelectric electroencephalograms, and with absent-evoked poten tials. Finally, the ethical aspects of these findings, as they relate to an encephaly and organ transplantation, are discussed, (C) 2000 by Elsevier Sc ience Inc. All rights reserved.