Presentation
History of involuntary eye movements in all directions since three months of age. Ophthalmology evaluation showed optic nerve hypoplasia.
Patient Data
Hypoplasia of the bilateral optic nerves (involving the posterior part of the intra-orbital segment, intra-canalicular and intra-cranial segments) and the optic chiasm (best appreciated on coronal T2-weighted images).
Absent septum pellucidum with associated mild flattening of the anterior horns of the lateral ventricles with point-down appearance of the inferior aspect of the anterior horn: (best appreciated on coronal T2-weighted images).
Ectopic posterior pituitary gland: (best appreciated on sagittal T1-weighted images as a high signal focus seen at the median eminence).
Selected image of coronal T2-weighted sequence showed the normal olfactory nerves (within the red cycles).
Selected image of coronal T2-weighted sequence showed point-down appearance of the anterior horns of the bilateral lateral ventricles (red arrows).
Selected image of sagittal T1-weighted sequence showed the ectopic posterior pituitary gland at the roof of the third ventricle (red circle).
Case Discussion
This is a case where most of the septo-optic dysplasia features are nicely demonstrated.
Hypoplastic optic nerves and chiasm, absence septum pellucidum with resultant typical configuration of the anterior horns of the lateral ventricles, along with ectopia of the posterior pituitary gland. On the other hand, the globes are intact and the olfactory bulbs are present with no evidence of associated parenchymal abnormality.
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