BRAIN-LESIONS OF FETAL ONSET IN ENCEPHALOPATHIC INFANTS WITH NONIMMUNE HYDROPS-FETALIS

Nonimmune hydrops fetalis (NIHF) comprised 79% (45/57) of all infants with hydrops fetalis at our institution over a 6-year period. Thirty-s even infants with NIHF were liveborn. One or more electroencephalogram s were performed on 40% of liveborn infants (15/37); the majority (87% ) were moderately to markedly abnormal, including burst suppression, l ack of background, multifocal sharp waves, excessive discontinuity, an d disorganization reflecting significant neonatal encephalopathies. Po stmortem neuropathologic examinations were performed in 86% of infants with NIHF who died or were stillborn, 81% of whom demonstrated intrau terine brain insults including microcalcifications, cerebral and/or ce rebellar hypoplasia, microcephaly, encephalomalacia, cavitary lesions, astrocytosis, polymicrogyria, and severe neuronal loss. Cranial ultra sonography failed to document the diverse pathologic lesions that were later noted on postmortem examination, Ten infants survived the neona tal period, but 6 were neurologically abnormal at the time of discharg e. Infants with NIHF are at risk for antepartum brain injury, and elec troencephalographic abnormalities reflect in part a fetal brain disord er. A prospective clinical study is needed to fully assess the prevale nce, incidence, spectrum of central nervous system involvement, contri bution of intrapartum and neonatal stress, and long-term outcome in su rviving infants with NIHF.