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Primary Empty Sella Syndrome: an Unusual Presentation of Panhypopituitarism

Kalina Naydenova, Hristo Milev, Katya Todorova, Beatrice Pancheva, Nadya Docheva

Abstract

Introduction: This study is aimed at reporting an rare case of an empty pituitary fossa clinically manifested by severe endocrine dysfunction in a 50-year-old male patient, which was radiologically confirmed years after the initial onset of symptoms.

Materials and Methods: A detailed medical history was obtained, medical documents from previous hospitalisations were reviewed including data from medical procedures, blood and  hormonal tests, and imaging results. The chief complaints of the patient included growth failure and cryptorchidism in his childhood along with fatigue, sleep deprivation and infertility which persist in his adulthood and recent abdominal pain in combination with gastrointestinal disorder.

Results: The diagnosis was based on the radiological evidence of cerebrospinal fluid within the sella turcica accompanied by the presence of ectopic hypoplastic pituitary gland in the region of the tuber cinereum. The hormonal test results correlated with the clinical presentation of consequent hyposomatotropism, hypogonadism, hypothyroidism and hypocorticism. Hyperprolactinemia, low cortisol levels with inverted rhythm of secretion and hypercholesterolemia were also present in this case.

Conclusion: Although most commonly empty sella is an incidental finding in what appears to be asymptomatic patients, this report shows a clinically significant case when diagnosis is delayed resulting in insufficient initial treatment. So despite being rare, when present, it may have severe complications if left untreated such as increased intracranial pressure, cerebrospinal fluid rhinorrhea and vision impairment. These are reasons why patients with Primary empty sella syndrome should undergo endocrine, neurological, and ophthalmological evaluation on presentation. Cardiac evaluation is also of vital importance in cases of hypopituitarism as morbidity in this group of patients is increased.


Keywords

primary empty sella, hypopituitarism, diagnosis


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