Abstract
BackgroundPrimary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. We intended to review the clinical management and hormonal profile of patients with PES.MethodsThe study included ten-year retrospective analysis of registry containing PES cases in the period 2007 to 2017, from a single tertiary care center. The keyword ‘primary empty sella’ was used to retrieve patient details from the radiology unit. The clinical and biochemical profile of PES patients was analyzed. Case management of PES patients and their rate of referral to endocrinologists was explored.ResultsThe registry had 765 cases with a male: female ratio of 1:3.8 suggesting female predominance by almost four times. Although not significant, the onset of disease was earlier for males [Mean ± standard deviation (SD) (46.7 years ±17.3 vs 48.8 years±14.1), p = 0.110]. Almost 79% of the cases were found as an incidental finding during Magnetic Resonance Imaging. Of the total PES cases, only 20% were referred to the endocrinologists and the rest were handled by general physicians. Only 1–2.5% of the cases were evaluated for gonadal, growth and adrenal hormones by the general physicians. The hormonal evaluation by the endocrinologists was also found to be sub-optimal. Headache and visual disturbances were the most common presenting complaints followed by menstrual abnormalities. Endocrine abnormalities like thyroid dysfunction, hyperprolactinemia, hypogonadism and hypocortisolism were highly prevalent among those assessed.ConclusionThere is a gross under-evaluation of hormonal assessment and minimal case-referral to Endocrinologists. PES is associated with varying degrees of hormonal dysfunction, and hence early assessment and management is needed. Establishing a standard protocol for diagnosis and case management is essential with the involvement of a multidisciplinary team consisting of endocrinologists, neurologists, primary care phys icians and ophthalmologists.
Highlights
Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans
The empty sella has been categorized as primary empty sella or secondary empty sella based on the etiology, which is mostly unclear while many hypotheses have been proposed
The findings of the study denote PES to be more than an incidental finding since high prevalence of co-existing endocrine abnormalities were found
Summary
Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. The congenital absence of diaphragm sellae is the only established etiology for PES, while other causes may include relative changes in intracranial pressure from the cerebrospinal fluid (CSF). The cause of empty sella is either primary (idiopathic) and most likely related to congenital defect in diaphragmatic sella or secondary (acquired) due to pituitary surgery, radiation and pituitary apoplexy. The most common clinical presentation of empty sella syndrome is persistent headache and hormonal deficiencies are rare as clinical presentation for that reason in our paper as in others the diagnosis of empty sella is incidental finding during the work up of neurological symptoms. Since hormonal deficiencies are rare and most likely acquired, the growth and the sexual development less likely to be affected
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