Abstract

Pituitary stalk interruption syndrome (PSIS) is a rare congenital syndrome characterized by a classical triad of interrupted pituitary stalk, ectopic posterior pituitary and hypoplasia or aplasia of anterior pituitary with varying degrees of pituitary hormone deficiency. It presents with hypoglycemia, prolonged jaundice, cryptorchidism and micropenis in neonates while short stature and delayed puberty are the main findings in older children and adults. The diagnosis of PSIS is made according to radiological findings. This report describes two cases with PSIS who were diagnosed at the ages of six and sixteen with diverse clinical findings.