Two cases of prenatal diagnosis of lissencephaly are presented in the
context of a series of 118 cases of prenatally diagnosed hydrocephalus
. Within this series there was one case of Walker-Warburg syndrome and
another of Miller-Dieker syndrome. It is stressed that the cases repo
rted here of ventriculomegaly diagnosed in utero show a very different
outcome from those in published studies of fetal hydrocephalus which
only deal with patients in whom the diagnosis was determined after bir
th. In those postnatal series there is a considerable selection bias,
and the fate of the fetuses reported here was much worse than in postn
atal series. Of the 118 fetuses 6 had fetal infections, 6 had chromoso
mal abnormalities, 26 had associated spina bifida, 64 fetuses had asso
ciated other anomalies, and only 28 had isolated hydrocephalus. Althou
gh it is difficult to determine the prognosis individually after prena
tal diagnosis of ventriculomegaly, the data presented here may be help
ful in counseling parents prenatally. The counseling should be perform
ed with the collaboration of obstetricians, pediatricians, surgeons, a
nd geneticists.