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.
MR imaging diagnosis of diencephalic-mesencephalic junction dysplasia in fetuses with developmental ventriculomegaly / Severino, M.; Righini, A.; Tortora, D.; Pinelli, L.; Parazzini, C.; Morana, G.; Accorsi, P.; Capra, V.; Paladini, D.; Rossi, A.. - In: AJNR, AMERICAN JOURNAL OF NEURORADIOLOGY. - ISSN 0195-6108. - 38:8(2017), pp. 1643-1646. [10.3174/ajnr.A5245]
MR imaging diagnosis of diencephalic-mesencephalic junction dysplasia in fetuses with developmental ventriculomegaly
Paladini D.;
2017
Abstract
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.File | Dimensione | Formato | |
---|---|---|---|
Paladini 4.pdf
accesso aperto
Licenza:
Accesso privato/ristretto
Dimensione
942.6 kB
Formato
Adobe PDF
|
942.6 kB | Adobe PDF | Visualizza/Apri |
I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.