Holoprosencephaly is a congenital malformation of the face and brain, which is caused by a defect of cleavage. It can be divided into alobar, semilobar, and lobar types according to the severity of its involvement. In this paper, we report two cases of semilobar holoprosencephaly. One was born full term with chromosomal abnormality. His mother had vaginal bleeding during the first trimester. Patient died at the age of 5 months and autopsy was made. The other one was born at the age of 34 weeks by caesarean section because of hyclrocephalus. Thickening of the cerebral mantle was found after ventriculo-peritoneal shunt insertin.
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