MR Imaging Diagnosis of Diencephalic-Mesencephalic Junction Dysplasia in Fetuses with Developmental Ventriculomegaly

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 oth...

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Bibliographic Details
Published in:American journal of neuroradiology : AJNR 2017-08, Vol.38 (8), p.1643-1646
Main Authors: Severino, M, Righini, A, Tortora, D, Pinelli, L, Parazzini, C, Morana, G, Accorsi, P, Capra, V, Paladini, D, Rossi, A
Format: Article
Language:English
Subjects:
Abnormalities
Adult
Brain
Diagnosis
Diencephalon
Diencephalon - abnormalities
Diencephalon - diagnostic imaging
Dysplasia
Female
Fetus
Fetuses
Gestational Age
Humans
Hydrocephalus
Hydrocephalus - congenital
Hydrocephalus - diagnostic imaging
Image detection
Magnetic Resonance Imaging
Mesencephalon
Mesencephalon - abnormalities
Mesencephalon - diagnostic imaging
Nervous System Malformations - diagnostic imaging
Neuroimaging
Pediatrics
Pregnancy
Pregnancy Trimester, Second
Prenatal Diagnosis
Surgery
Ventricle
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Description
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.
ISSN:0195-6108
1936-959X
DOI:10.3174/ajnr.A5245