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      Clinical presentation, evaluation and case management of primary empty sella syndrome: a retrospective analysis of 10-year single-center patient data

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          Abstract

          Background

          Primary 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.

          Methods

          The 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.

          Results

          The 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.

          Conclusion

          There 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.

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          Most cited references19

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          DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review.

          Primary empty sella (PES) is characterized by the herniation of the subarachnoid space within the sella, which is often associated with variable degrees of flattening of the pituitary gland in patients without previous pituitary pathologies. PES pathogenetic mechanisms are not well known but seem to be due to a sellar diaphragm incompetence, associated to the occurrence of upper sellar or pituitary factors, as intracranial hypertension and change of pituitary volume. As PES represents in a majority of cases, a neuroradiological findings without any clinical implication, the occurrence of endocrine, neurological and opthalmological symptoms, due to the above describes anatomical alteration, which delineates from the so called PES syndrome. Headache, irregular menses, overweight/obesity and visual disturbances compose the typical picture of PES syndrome and can be the manifestation of an intracranial hypertension, often associated with PES. Although hyperprolactinemia and growth hormone deficit represent the most common endocrine abnormalities, PES syndrome is characterized by heterogeneity both in clinical manifestation and hormonal alterations and can sometime reach severe extremes, as occurrence of papilledema, cerebrospinal fluid rhinorrhea and worsening of visual acuity. Consequently, a multidisciplinary approach, with the integration of endocrine, neurologic and ophthalmologic expertise, is strongly advocated and recommended for a properly diagnosis, management, treatment and follow-up of PES syndrome and all of the related abnormalities.
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            Idiopathic intracranial hypertension: the validity of cross-sectional neuroimaging signs.

            The aim of this study was to evaluate the accuracy of previously reported neuroimaging signs in establishing or excluding the diagnosis of idiopathic intracranial hypertension (IIH). In a retrospective study, 30 patients with confirmed IIH and 56 controls were evaluated with brain magnetic resonance imaging. All examinations were evaluated in a blinded fashion by three neuroradiologists for the presence or absence of the 'traditional' signs of IIH: empty sella turcica, deformation of the pituitary, slit-like ventricles, 'tight' subarachnoid spaces, flattening of the posterior globe, protrusion of the optic nerve, enhancement of the optic nerve head, distension of the optic nerve sheath and vertical tortuosity of the optic nerve. Optic nerve protrusion and enhancement, slit-like ventricles and tight cerebrospinal fluid spaces were not significantly associated with IIH (P>0.05). Posterior globe flattening, optic nerve sheath distension, optic nerve tortuosity, pituitary deformity and empty sella turcica were significantly associated with IIH (P<0.05). However, most of these are not helpful in a clinical setting, with the exception of posterior globe flattening. This is the only sign that, if present, strongly suggests the diagnosis of IIH (specificity 100%, 95% CI 93.6% to 100%; sensitivity 43.5%, 95% CI 27.3% to 60.8%; positive likelihood ratio 49.7). The majority of the reported signs for IIH on cross-sectional imaging are not helpful in establishing or excluding the diagnosis of IIH, and are of no value in the clinical setting. Flattening of the posterior aspect of the globe is the only sign that, if present, is suggestive of the diagnosis of IIH.
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              Primary empty sella (PES): a review of 175 cases.

              The term primary empty sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in patients with no history of pituitary tumor, surgery or radiotherapy. To retrospectively assess clinical features, radiological findings and the biochemical endocrine function from the records of 175 patients with a diagnosis of PES. One hundred seventy-five patients (150 females) were studied. The mean age at diagnosis was 48.2 ± 14 year. Most diagnoses were made by magnetic resonance imaging (n = 172). In most patients, the pituitary function was assessed by basal pituitary hormones measurements. Pituitary scans were ordered for different reasons: headache (33.1 %), endocrine disorders (30.6 %), neurological symptoms (12.5 %), visual disturbances (8.75 %), abnormalities on sella turcica radiograph (8.75 %) and others (6.25 %). Multiple pregnancies were observed in 58.3 % of women; headaches, obesity, and hypertension were found in 59.4, 49.5, and 27.3 % of the studied population, respectively. Mild hyperprolactinemia (<50 ng/ml) was present in 11.6 % of women and 17.3 % of men. Twenty-eight percent of our patients had some degree of hypopituitarism. In the male population, hypopituitarism represented 64 % of cases, whereas it accounted for 22 % of all females. PES seems to be more commonly found in middle-aged women, with a history of multiple pregnancies. In most patients, PES was discovered as an incidental finding on imaging studies, while in almost a quarter of patients PES was found during the diagnostic evaluation of anterior pituitary deficiency, which was more common in men.
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                Author and article information

                Contributors
                aishahekhzaimy@hotmail.com , aaekhzaimy@ksu.edu.sa
                mujammami@yahoo.com
                shabstharkar@yahoo.com
                alansary.m.a@gmail.com
                daadotaibii@gmail.com
                Journal
                BMC Endocr Disord
                BMC Endocr Disord
                BMC Endocrine Disorders
                BioMed Central (London )
                1472-6823
                17 September 2020
                17 September 2020
                2020
                : 20
                : 142
                Affiliations
                [1 ]GRID grid.56302.32, ISNI 0000 0004 1773 5396, Endocrinology and Diabetes Unit, Department of Medicine, College of Medicine and King Saud University Medical City, , King Saud University, ; Riyadh, Saudi Arabia
                [2 ]GRID grid.56302.32, ISNI 0000 0004 1773 5396, Prince Sattam Chair for Epidemiology and Public Health Research, Department of Family and Community Medicine, College of Medicine, , King Saud University, ; Riyadh, Saudi Arabia
                Author information
                http://orcid.org/0000-0002-8024-0631
                Article
                621
                10.1186/s12902-020-00621-5
                7495892
                32943019
                be131b96-cd5d-441f-9047-dfe0b4e0a877
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 1 September 2019
                : 6 September 2020
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2020

                Endocrinology & Diabetes
                case management,empty sella syndrome,hormone assessment,saudi arabia
                Endocrinology & Diabetes
                case management, empty sella syndrome, hormone assessment, saudi arabia

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