Primary empty sella syndrome with micropenis and hyperprolactinemia

Abstract

Empty sella syndrome is a rare entity in paediatrics. A case is presented in a 13-year old boy who was referred for a micro-penis. He was born at term by breech delivery and recovered rapidly from a neonatal subarachnoid hemorrhage. Psychomotor development was normal. Physical examination showed a micropenis with normal prepubertal testes and a delayed growth with height at - 3 standard deviations. There was no sign of puberty and no gynecomastia or galactorrhea. Endocrinological studies indicated growth hormone, TSH, ACTH and gonadotropin deficiencies contrasting with elevated basal plasma levels of prolactin (260 μg/1) which failed to increase with TRH stimulation. Skull roentgenograms, tomography of the sella turcica and computerized tomographic brain scan were normal. There were no abnormalities of the fundi and visual fields. Diagnosis of empty sella was made on pneumoencephalogram showing the absence of a sella mass. Treatment with bromocryptine led to a normalisation of the plasma prolactin levels but failed to modify the other hormonal pituitary dysfunctions. Increased statural growth was obtained under substitutive therapy with human growth hormone, thyroxine and hydrocortisone.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD