SEPTO-OPTIC DYSPLASIA WITH AN ARACHNOID C YST

A female infant presenting with serious hypoglycemia and lazy feeding was diagnosed as suffering from septo-optic dysplasia (SOD). SOD is in dicated by optic nerve hypoplasia, agenesis of the septum pellucidum a nd a variety of endocrinologic abnormalities. The characteristics in o ur case were diabetes insipidus, hypothyreosis and adrenal cortical in sufficiency. Neuroradiologic evaluation revealed an arachnoid cyst in the parieto-occipital region. To our knowledge this is the first time that this finding has been found in a patient with SOD. The etiology o f this midline defect is unknown. An autosomal-recessive trait appears to be possible because of parental consanguinity. Early diagnosis of this syndrome is extremely important because of the need for hormonal substitution.