Septo-optic dysplasia

Septo-optic-dysplasia-Jan-2013.jpg

Image 1

Clinical History: 
A five year-old boy with hydrocephalus and pituitary dysfunction

Diagnosis:
Septo-optic dysplasia

MR Technique: 
Axial and coronal SE T1-weighted images are presented, with TR/TE = 400-458/10, obtained at 1.5 T on a Siemens Avanto MR system. Scan times were 2:41 and 3:07, with a slice thickness of 5 mm.

Imaging Findings:
There is gross dilation of the lateral and third ventricles. The septum pellucidum is noted to be absent. The optic chiasm (seen on the coronal image) and proximal portions of the optic nerves (not depicted) are noted to be small. The infundibulum is small. All are characteristic findings in this entity, which is generally diagnosed early in life, with endocrine abnormalities seen in 60%.