All issues > Volume 35(6); 1992

Case Report

J Korean Pediatr Soc. 1992;35(6):809-815. Published online June 15, 1992.

A Case of Congenital Hypopituitarism Presenting Ectopic Pituitary Gland and Pituitary Stalk Transection Identified by Magnetic Resonance Imaging

Do Seong DS Kim¹, Kyung Eun KE Oh¹, Duk Hi DH Kim¹, Myung Joon MJ Kim²

¹Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
²Department of Radiology, College of Medicine, Yonsei University, Seoul, Korea

Abstract

The syndrome of congenital hypopitutarism is a rare but well recognized entity. The typical clinical picture consists of presentation in infanncy with hypoglycemia, microgenitalia, and evidence of multiple anterior pituitary hormone deficiencies, but not diabetes inspidus. Recently, magnetic resonance imaging has disclosed a consistent abnormality in many patients with congenital hypopituitarism as well as idiopathic hypopituitarism characterized by an adenohypophysis of varying volume, attenuation or transection of the pituitary stalk, absence of the usual intrasellar location of the high intensity signal of the posteior pituitary, and the presence of such an image at or below the median eminence of the hypothalamus, considered an ectopic neurohypophysis. We have recently seen a case of congenital hypopituitarism in a 34/12-year-old male patient with microgenitalia and short stature. The endocrinological studies revealed growth hormone and gonadotropin-releasing hormone deficiencies. The magnetic resonance imaging showed characteristic findings of congenital hypopituitarism. The patient was followed-up with growth hormone therapy.

Keywords :Congenital hypopituitarism, Magnetic resonance imaging, Ectopic posterior pituitary gland