Korean J Med.  2013 Oct;85(4):420-424.

Hypothalamic Hypopituitarism Caused by Pituitary Stalk Dysgenesis

• Lee SJ
• Yoon HJ
• Cho AR
• Um YJ
• Park KY
• Lim DM
• Kim BJ

Abstract

Functional defects of the pituitary gland are a rare cause of pubertal delay. The pituitary stalk is an important structure that connects the hypothalamus and pituitary gland. A defect in fusion of the pituitary stalk and anterior pituitary gland will block the function of the anterior pituitary gland. A 28-year-old man was referred to our clinic with poorly developed secondary sexual characteristics. He had undeveloped facial, axillary, and pubic hair and was Tanner stage I. Laboratory tests gave random serum testosterone < 0.025 ng/mL, luteinizing hormone (LH) < 0.1 mIU/mL, follicle-stimulating hormone (FSH) 0.626 mIU/mL, thyroid-stimulating hormone (TSH) 6.85 microIU/mL, and fT4 6.96 pmol/L. Sella magnetic resonance imaging (MRI) showed no pituitary stalk enhancement. The response in the combined pituitary function test revealed multiple hormonal defects, while the TSH response to thyrotropin-releasing hormone (TRH) was exaggerated and delayed. Therefore, we concluded that pituitary stalk dysgenesis had led to hypothalamic-type panhypopituitarism.

Keyword

Delayed puberty; Hypothalamic hypopituitarism; Pituitary stalk dysgenesis

MeSH Terms

Adult
Follicle Stimulating Hormone
Hair
Humans
Hypopituitarism
Hypothalamus
Luteinizing Hormone
Magnetic Resonance Imaging
Pituitary Function Tests
Pituitary Gland
Pituitary Gland, Anterior
Puberty, Delayed
Testosterone
Thyrotropin
Thyrotropin-Releasing Hormone
Follicle Stimulating Hormone
Luteinizing Hormone
Testosterone
Thyrotropin
Thyrotropin-Releasing Hormone