ESPE Abstracts

Background: Various studies, mostly conducted in adults, evaluated the hormonal axis in primary empty sella (PES), and reported different forms of pituitary deficiencies. We report our 10-year experience of pediatric cases with PES, investigating the pituitary function, associated impairments, and responses to treatments.

Materials and Methods: We reviewed 10,560 cranial and 325 pituitary magnetic resonance imagings (MRIs) performed in our university hospital between January 2010 and December 2020. Cases with additional abnormal MRI findings, history of cranial surgery or radiotherapy, autoimmunity, long-term use of chemotherapeutic or immune-suppressive agents, or incomplete diagnostic evaluation were excluded. Clinical, radiological, and laboratory evaluations were recorded. The results are reported as median (interquartile range, minimum-maximum).

Results: The study included 16 patients [8 girls, 8 boys; median age 12.4 years (8.3, 4.3-17)]. The median size of the pituitary was 2 mm (0.8, 1.2-3). Based on age-dependent pituitary height measurements, fourteen (88%) subjects showed pituitary gland hypoplasia. Five cases presented due to short stature, two had both pubertal delay and short stature, and one had pubertal delay. Eight cases had presented with neurological symptoms including headache, tinnitus, tics, and dizziness. Two of the seven short subjects had normal growth velocities for age with IGF-1/IGFBP-3 levels >0 SD score. The remaining five short subjects had growth deceleration and/or target height discrepancies with low to normal IGF-1/IGFBP-3 levels. All these patients failed to achieve an adequate growth hormone (GH) peak in GH stimulation tests; thus, GH therapy was initiated. The patients with pubertal delay showed normal pubertal responses to gonadotropin-releasing hormone stimulation test. Five of the patients had a baseline cortisol level below 10 mcg/dL. All of them showed normal cortisol responses to stimulation tests. None of the patients had hyper- or hypoprolactinemia, hypothyroidism, or diabetes insipidus. There was no statistically significant association between the size of pituitary gland and the severity of hypopituitarism (P = 0.472).

Conclusion: The high incidence of pituitary dysfunctions ascertain that this entity should not be considered as a normal variant, instead should be evaluated carefully with appropriate basal and dynamic hormonal testing to identify the varying hormonal dysfunctions.