Septo-optic dysplasia associated with polyendocrine dysfunction

Septo-optic dysplasia (SOD) is a rare congenital disease that has been described in association with hypothalamic dysfunction. A case of polyendocrine dysfunction is described in a 22 year-old white female with septo-optic dysplasia. Findings included atrophic optic nerves, a cavum septum pellucidum and a small pituitary gland detected by magnetic resonance imaging. The patient had central diabetes insipidus, Hashimoto's thyroiditis, and gestational diabetes mellitus. A review of the literature is presented. A careful and detailed endocrine evaluation is needed in patients with SOD.