Incidental discovery of subtle-variant septooptic dysplasia in an adult with headaches: a novel case with pathophysiologic implications

Abstract

Septooptic dysplasia (SOD) is a congenital central nervous system malformation syndrome classically associated with the triad of agenesis of the septum pellucidum, optic nerve hypoplasia, and pituitary abnormalities. It has been suggested that SOD may result from in utero vascular insults. We present the case of an adult male with personal and family histories of intracranial vascular pathology in whom SOD was incidentally discovered, and we describe how the specific abnormalities in this case could be related to vascular pathology.