Primary Empty Sella and Rieger's Anomaly of the Anterior Chamber of the Eye

Abstract

EXTENSION of the subarachnoid space into the sella turcica, in the absence of prior radiotherapy or surgery, is referred to as primary empty sella. In most patients with this anomaly, the sella is diffusely enlarged and pituitary function is normal.^{1 2 3 4} Of major clinical importance in primary empty sella is the differentiation of this condition from a pituitary tumor. Most patients with the primary-empty-sella syndrome have been obese, middle-aged women.^{1 2 3 4} The condition is rarely familial,^{5 , 6} and it is infrequently observed in children.^{5 6 7} This report describes a kindred in which primary empty sella was transmitted as an autosomal-dominant trait in association with . . .