TRILOBAR HOLOPROSENCEPHALY (TRIPROSENCEPHALY) - A UNIQUE TYPE OF CEREBRAL MALFORMATION

Little is known about the neuropathology of the median facial cleft sy ndrome, which presents as a combination of a wide range of teratologic al manifestations. We report a unique type of cerebral malformation co mbined with the median facial cleft, syndrome in a 7-day-old female in fant with malformations of toes and fingers, hypertelorism and a media n cleft: nose, as well as a frontally protruding, dorsomedian hornlike cele. At autopsy, the cranium presented facial clefts and bony defect s, resulting in partitioning of the anterior cranial fossa into three compartments, The brain had malformative features of lobar holoprosenc ephaly combined with tripartition of frontal lobes, including an encep halomeningocystocele originating from a right accessory frontal lobe.