Abstract

Two proven cases of lobar holoprosencephaly with hydrocephalus and on likely but unproven case were studied in utero by sonography. Characteristic sonographic features were defind which corresponded to known anatomic and CT features of this entity. These include incomplete anterior falx, absence of the septum pellucidum and corpus callosum, apparent marked hydrocephalus with little parietoccipital mantle (representing in part a “dorsal” cyst), abnormal thalami, and superior and anterior blunting of the frontal horns. Differential diagnostic considerations for lobar holoprosencephaly with hydrocephalus include simple obstructive hydrocephalus (aqueductal stenosis, etc.) and various cystic intracranial malformations. Lobar holoprosencephaly is the least severe of a spectrum of cerebral malformations resulting from-incomplete division of the embryonic prosencephalon into two hemispheres.

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