ESPE Abstracts

Introduction: Empty sella, is a radiologic finding in which the sella turcica appears empty due to cerebral spinal fluid (CSF) within the subarachnoid space herniating into the sella turcica. Subsequently, the pituitary gland contained in the sella turcica is compressed and flattened, and the pituitary stalk is stretched by the CSF, which fills the space. It’s usually associated with growth hormone (GH) restriction which is clinically imprinted with a decrease in the annual growth rate < 4cm. Specifically, it has been noticed that 27% of children with GH restriction, presented with empty sella in the Magnetic Resonance Imaging (MRI). Indeed, GH restriction, among the pituitary deriving hormones, is the most common hormonal disorder in terms of empty sella syndrome. In literature it is suggested that in 5-58% of the children with GH restriction, primary empty sella co-exists. The purpose of this report is to describe a patient with empty sella syndrome, that clinically developed at puberty.

Case report: A 14-year-old male adolescent patient, who had been followed up at the pediatric endocrinology clinic since the age of 9, with normal physical growth, presented at the age of 11-12 with a drop in the annual growth rate. This observation was initially attributed to constitutive growth retardation and puberty. (the father had also presented a similar delay). However, at the age of 14, still he didn’t show any clinical or laboratory findings of puberty induction. Thus, in terms of investigation, the following tests were performed: MRI of the pituitary gland, Luteinising Hormone Releasing Hormone (LHRH) test, Ultrasound of the kidneys, ureters, and bladder. From the carried-out investigation, the only finding was from the MRI of the pituitary gland, which showed a gentle submerge of the supraspinatus cistern within the sella turcica. Based on the above and the constantly low values of Insulin-like Growth factor 1 (IGF-1) for the patient’s age, two GH stimulation tests were carried out, (having previously a testosterone priming) and both were abnormal.

Conclusion: The reduction of the annual growth rate as well as the short stature, are conditions that need detailed investigation. In our patient, from the laboratory and radiology tests, results in favor of a rare syndrome came out. Subsequently, in this case, growth hormone replacement therapy is indicated as well as a close follow-up in the pediatric endocrinology clinic, for a possible restriction in other pituitary gland hormones.