RT Journal Article
SR Electronic
T1 MR Imaging Diagnosis of Diencephalic-Mesencephalic Junction Dysplasia in Fetuses with Developmental Ventriculomegaly
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1643
OP 1646
DO 10.3174/ajnr.A5245
VO 38
IS 8
A1 Severino, M.
A1 Righini, A.
A1 Tortora, D.
A1 Pinelli, L.
A1 Parazzini, C.
A1 Morana, G.
A1 Accorsi, P.
A1 Capra, V.
A1 Paladini, D.
A1 Rossi, A.
YR 2017
UL http://www.ajnr.org/content/38/8/1643.abstract
AB SUMMARY: Diencephalic-mesencephalic junction dysplasia is a rare malformation characterized by a poorly defined junction between the diencephalon and the mesencephalon, associated with a characteristic butterfly-like contour of the midbrain (butterfly sign). This condition may be variably associated with other brain malformations, including callosal abnormalities and supratentorial ventricular dilation, and is a potential cause of developmental hydrocephalus. Here, we have reported 13 fetuses with second-trimester obstructive ventriculomegaly and MR features of diencephalic-mesencephalic junction dysplasia, correlating the fetal imaging with available pathology and/or postnatal data. The butterfly sign can be clearly detected on axial images on fetal MR imaging, thus allowing for the prenatal diagnosis of diencephalic-mesencephalic junction dysplasia, with possible implications for the surgical management of hydrocephalus and parental counseling.DMJdiencephalic-mesencephalic junction