DOI: https://dx.doi.org/10.18203/2320-6012.ijrms20223107
Published: 2022-11-25

Primary empty sella syndrome presenting as adrenal insufficiency and without raised intracranial tension

Ishank Gupta, Vinay Panchalwar, Aman Chabra

Abstract


Empty sella syndrome (ESS) based on pathophysiology can be either primary empty sella syndrome (PES) or secondary empty sella syndrome (SES). Patients of PES have no known pituitary pathology and have varying degrees of pituitary gland flattening. We present a 65-year-old female who came with complaints of generalised swelling over the body, generalised weakness, altered sensorium and low-grade fever. Patient had history of ischaemic heart disease 3 years back without any other known co-morbidities. Patient presented in hypoglycaemia and hypotension with facial puffiness and oedema feet with basal crepitations indicating fluid overload state. Patient was stabilised and evaluated. Patient was found to have hyponatremia and urinary tract Infection and associated acute kidney injury. Patient’s morning cortisol and adrenocorticotropic hormone (ACTH) levels were also low with associated low insulin-like growth factor 1 (IGF-1) and normal thyroid stimulating hormone (TSH) levels but low T3 and low T4 levels. Patient’s magnetic resonance imaging (MRI) brain was done which was suggestive of PES. The cause of empty sella remained unclear but the adrenal insufficiency was treated with hydrocortisone and the patient became asymptomatic and was discharged subsequently.


Keywords


Adrenal insufficiency, Cortisol, ACTH, IGF-1, TSH, Primary empty sella syndrome

Full Text:

PDF

References


Chiloiro S, Giampietro A, Bianchi A, De Marinis L. Empty sella syndrome: Multiple endocrine disorders. Handb Clin Neurol. 2021;181:29-40.

Ucciferro P, Anastasopoulou C. Empty Sella. In: StatPearls. Treasure Island (FL): StatPearls Publishing. 2022.

RNass R, Helm KD, Evans WS. Chapter 21 - Physiological and Pathophysiological Alterations of the Neuroendocrine Components of the Reproductive Axis, Editor(s): Strauss JF, Barbieri RL. Yen & Jaffe's Reproductive Endocrinology (Seventh Edition). W.B. Saunders. 2014;439-84.

Chiloiro S, Giampietro A, Bianchi A, Tartaglione T, Capobianco A, Anile C, De Marinis L. Diagnosis of endocrine disease: Primary empty sella: a comprehensive review. Eur J Endocrinol. 2017;177(6):R275-85.

Zagardo MT, Cail WS, Kelman SE, Rothman MI. Reversible empty sella in idiopathic intracranial hypertension: an indicator of successful therapy? AJNR Am J Neuroradiol. 1996;17(10):1953-6.

Miljic D, Pekic S, Popovic V. Empty Sella. In: Feingold KR, Anawalt B, Boyce A, et al, editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000.

Colao A, Cotta OR, Ferone D, Torre ML, Ferraù F, Di Somma C, et al. Role of pituitary dysfunction on cardiovascular risk in primary empty sella patients. Clin Endocrinol (Oxf). 2013;79(2):211-6.

Auer MK, Stieg MR, Crispin A, Sievers C, Stalla GK, Kopczak A. Primary empty sella syndrome and the prevalence of hormonal dysregulation. Dtsch Arztebl Int. 2018;115(7):99-105.

Del Monte P, Foppiani L, Cafferata C, Marugo A, Bernasconi D. Primary "empty sella" in adults: endocrine findings. Endocr J. 2006;53(6):803-9.

Sachdev Y, Evered DC, Appleby A, et al The empty sella syndrome. Postgrad Med J. 1976;52:703-5.

Guitelman M, Garcia Basavilbaso N, Vitale M, Chervin A, Katz D, Miragaya K, et al. Primary empty sella (PES): a review of 175 cases. Pituitary. 2013;16(2):270-4.

De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A. Primary empty sella. J Clin Endocrinol Metab. 2005;90(9):5471-7.

Giustina A, Aimaretti G, Bondanelli M, Buzi F, Cannavò S, Cirillo S, et al. Primary empty sella: Why and when to investigate hypothalamic-pituitary function. J Endocrinol Invest. 2010;33(5):343-6.

Faje A, Tritos NA, Swearingen B, Klibanski A. Neuroendocrine disorders: pituitary imaging. Handb Clin Neurol. 2016;136:873-85.

Doroftei NA, de Rudder C, de Visscher N, Hanon F. Isolated ACTH deficiency in a patient with empty sella as revealed by severe hyponatremia. Acta Clin Belg. 2016;71(6):451-4.