PT  - JOURNAL ARTICLE
AU  - M. Severino
AU  - A. Righini
AU  - D. Tortora
AU  - L. Pinelli
AU  - C. Parazzini
AU  - G. Morana
AU  - P. Accorsi
AU  - V. Capra
AU  - D. Paladini
AU  - A. Rossi
TI  - MR Imaging Diagnosis of Diencephalic-Mesencephalic Junction Dysplasia in Fetuses with Developmental Ventriculomegaly
AID  - 10.3174/ajnr.A5245
DP  - 2017 Aug 01
TA  - American Journal of Neuroradiology
PG  - 1643--1646
VI  - 38
IP  - 8
4099  - http://www.ajnr.org/content/38/8/1643.short
4100  - http://www.ajnr.org/content/38/8/1643.full
SO  - Am. J. Neuroradiol.2017 Aug 01; 38
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