RESUMO
OBJECTIVE: Although advances in neuroradiologic imagings have enabled us to diagnose intrauterine central nervous system(CNS) anomalies, it's management remains difficult because the natural history and postnatal outcome were not well understood. This study is undertaken to clarify the natural history and postnatal outcome of prenatally diagnosed CNS anomaly. METHODS: Between January 1999 and October 2002 there were 18 cases of a fetus with a CNS anomaly prenatally diagnosed by ultrasonography. In six cases, intrauterine magnetic resonance(MR) image was undertaken to confirm the findings when ultrasonography was inconclusive. In this prospective follow-up study the authors have applied the "perspective classification of congenital hydrocephalus(PCCH)" proposed by Oi et al. RESULTS: Hydrocephalus was diagnosed in three cases at PCCH stage I, in six cases at stage II, in 9 cases at stage III. In 8 cases, termination of pregnancy were performed due to severe CNS anomalies. Among these cases, hydrocep halus was diagnosed in 3 cases(100%) at stage I, in 3 cases(50%) at stage II, in 2 cases(22.2%) at stage III. Out of these 8 cases, 5 cases underwent autopsy following parental consent. Of the 10 cases who were delivered, 4(40%) patients were treated. In these 4 cases, hydrocephalus was diagnosed at PCCH stage III(3 fetuses survived, 1 fetus died at 6 months after birth). CONCLUSION: The postnatal outcome is significantly poor in the fetuses with CNS anomaly diagnosed in the early gestation. So it is important to diagnose the fetal CNS anomalies in the early stage of gestation and follow up periodically.