Abstract

A 3 year old female child came to ophthalmology OPD at regional eye hospital kurnool presented with history of difficulty in fixating of both eyes.On examination the patient had horizontal nystagmus with no fixing and following of light.The pupillary reactions and anterior segment examination were normal.Fundus examination in both eyes revealed small optic disc with double ring sign which is suggestive of optic nerve hypoplasia.Magnetic resonance imaging (MRI)brain showed small/hypoplastic pituitary gland with diffuse thinning of pituitary stalk and corpus callosum dysgenesis with absent superior portion of interventricular septum.Based on examination and investigations diagnosis of de-morsier's syndrome was made.De-morsier's syndrome also known as septo-optic dysplasia characterised by optic nerve hypoplasia ,pituitary endocrine dysfunction and midline brain abnormalities as hypoplasia or absence of septum or corpus callosum or both.It is one of the causes of childhood blindness.