[New clinical phase in intrauterine diagnosis and therapeutic modalities of CNS anomalies].

Thirty one cases of prenatally diagnosed CSN malformations were encountered in our institutions between 1982 and 1988. There were 42 disclosed lesions including 24 hydrocephalus, 3 anencephaly, 3 myeloschisis, 3 holoprosencephaly, 3 encephalocele, 2 Dandy-Walker cyst, 1 hydroencephalodysplasia, 1 brain tumor (Teratoma), 1 sacrococcygeal teratoma and 1 sacral agenesis. The gestational age at diagnosis ranged from 24 to 40 weeks of gestation (average 33.4 weeks). All were diagnosed by ultrasonography, and either MRI or whole body CT was additionally performed to evaluate the morphology in ten patients. MRI was a definitely useful diagnostic tool to evaluate the intracranial morphology, whereas whole body CT was much superior to other diagnostic imagings to visualize the condition of cranium or cranial sutures. Using these diagnostic procedures the type of hydrocephalus, was able to be identified, according to the major categories, in the individual cases. Spine morphology, however, was not detected n such high quality by MRI. Ultrasonography combined with application of whole body CT, with or without MRI, may be the best method now available to diagnose spina bifida. However, at the present time, this cannot be done until after 24 weeks of gestational age. It is our strong impression that such diagnostic technique for fetal CNS malformations will be improved, and selection of therapeutic modalities will be decided in the prenatal period before birth in the near future. It will be a dramatic change in the clinical historical phase in the treatment of CNS malformations, but establishment of therapeutic regimen along with more precise analysis of natural history, pathophysiology, postnatal outcome and ethical aspects of these congenital anomalies is required.