septo-opticdysplasia with an anterior encephalocele and intact septum pellucidum: a case report

objective the diagnosis of de morsier syndrome or septo-optic dysplasia is made on the basis of the diagnosis of optic nerve hypoplasia. septo-optic dysplasia is defined by a variable combination of dysgenesis of midline brain structures including optic nerve hypoplasia and hypothalamic-pituitary dysfunction often associated with a wide variety of brain malformations of cortical development. the importance of direct ophthalmoscopy of optic nerve abnormalities is stressed, as well as of magnetic resonance imaging, which has become a guideline in the classification of  this syndrome this article reports a 19-year-old female with bilateral optic nerve  hypoplasia,anterior encephalocele and intact septum pellucidum. she was diagnosed with diabetes insipidus, short stature and the history of seizure.