J Korean Pediatr Soc.  1999 Dec;42(12):1746-1750.

A Case of Congenital Hypopituitarism with Anterior Pituitary Hypoplasia and Ectopic Posterior Pituitary Gland

• Son YJ
• Park HS
• Shim KS
• Choeh K
• Han TI

Abstract

The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic findings of congenital hypopituitarism can be different in each case. Congenital hypopituitarism is a rare disorder. The characteristic clinical features of the affected newborns are prolonged jaundice, persistent or recurrent hypoglycemia without hyperinsulinism and microphallus. Their genitalia are usually underdeveloped and sexual maturation may be delayed or absent. In adulthood, patients retain childish feature, short stature with normal body proportion. We experienced a case of congenital hypopituitarism in a 12-year-old female patient with short stature and delayed sexual maturation(Tanner stageI). The endocrinological studies revealed growth hormone, FSH, LH and TSH deficiencies. Magnetic resonance imaging indicated a hypoplastic anterior pituitary and an ectopic posterior pituitary gland located within the tuber cinereum of the hypothalamus.

Keyword

Congenital hypopituitarism; Anterior pituitary hypoplasia; Ectopic posterior pituitary gland

MeSH Terms

Brain
Child
Female
Genitalia
Growth Hormone
Humans
Hyperinsulinism
Hypoglycemia
Hypopituitarism*
Hypothalamus
Infant, Newborn
Jaundice
Magnetic Resonance Imaging
Pituitary Gland
Pituitary Gland, Anterior
Pituitary Gland, Posterior*
Sexual Maturation
Tuber Cinereum
Growth Hormone