Septo-Optic Dysplasia(De
Morsier Syndrome)
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Ultrasound
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Absent cavum septum pellucidum
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Hypoplastic optic nerves (visual impairment and
dysfunction of the pituitary- hypothalamic axis).
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Box-like frontal horns squared off dorsally and pointing
inferiorly at the caudothalamic groove
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Bulbous dilatation of the anterior recess of the third ventricle
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Dilatation of frontal and occipital horns of lateral ventricles
(temporal horns are not dilated as in hydrocephalus)
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Normal falx
and interhemispheric fissure
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Thin corpus callosum
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Less frequent features:
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Associated
with:
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Differential diagnosis: ·
Isolated absence
of the septum pellucidum – frontal horns usually
less squared and no additional anomalies are demonstrated. ·
Lobar holoprosencephaly – features may be identical.
Definitive diagnosis may only be made after birth by demonstrating optic
nerve hypoplasia. |
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Video clip of
absent cavum septum pellucidum |
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