[Congenital anomalies of the central nervous system--from the pediatric standpoint]

Congenital anomalies of the central nervous system frequently cause severe emotional, economic, and physical hardship for both the child and the family. Craniofacial and developmental abnormalities may cause social unacceptability. Congenital hydrocephalus may result in neonatal death or mental and motor disabilities. Frequent hospitalization of prolonged institutionalization may be necessary for the many surviving patients. With recent advances in the diagnostic techniques of amniocentesis, fetoscopy, ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI), an improved accuracy in the diagnosis of fetal abnormalities and in the determination of prognosis is possible in many instances. When confronted with the diagnosis of congenital anomalies of the central nervous system during the intra-uterine period, there are five options for management: 1) no intervention; 2) termination of the pregnancy; 3) allowing pregnancy to proceed to term; 4) induction of preterm delivery followed by postnatal intervention; and 5) intrauterine surgical intervention. However, in practice it may be very difficult to choose the best opinion. In order to help make such decisions, this paper reviewed the current understanding of the main congenital anomalies of the central nervous system. According to a 17 year survey of major congenital neurologic malformations in the USA, the incidence of major central nervous system anomalies (anencephaly, spina bifida and encephalocele) has been declining. This has also been found in Japan. In the USA an organization was established--The Medical Task Force on Anencephaly--for the purpose of offering guidelines on the ethical issues relating to anencephaly, especially in regard to the use of fetal organs in transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)