Prenatal MR findings of the middle interhemispheric variant of holoprosencephaly

We report a case of the middle interhemispheric variant of holoprosencephaly (MIH) with noncleavage of the posterior portion of the frontal lobes and the parietal regions in a fetus at 22 weeks' gestation. To our knowledge, this is the first case of the rare MIH variant to be diagnosed in utero by use of ultrafast MR imaging and one of the few such reports to document gross and microscopic pathologic findings. Neuroimaging results correlated with those of gross and microscopic pathologic specimens obtained from the stillborn child. We conclude that ultrafast MR imaging can accurately distinguish holoprosencephaly subtypes in utero, which may affect counseling of parents.