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 M. Severino
A1 A. Righini
A1 D. Tortora
A1 L. Pinelli
A1 C. Parazzini
A1 G. Morana
A1 P. Accorsi
A1 V. Capra
A1 D. Paladini
A1 A. Rossi
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