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Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome

Pituitary duplication is a rare congenital malformation. It has been mainly reported in the pediatric and neonatal population, with few reported cases in the adult population. In this case report, we discuss the presentation of an adult female patient with pituitary stalk duplication and primary emp...

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Autores principales: Alkhyeli, Fatima, Boharoon, Hessa, Almarzouqi, Abdulla
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10510566/
https://www.ncbi.nlm.nih.gov/pubmed/37736451
http://dx.doi.org/10.7759/cureus.43851
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author Alkhyeli, Fatima
Boharoon, Hessa
Almarzouqi, Abdulla
author_facet Alkhyeli, Fatima
Boharoon, Hessa
Almarzouqi, Abdulla
author_sort Alkhyeli, Fatima
collection PubMed
description Pituitary duplication is a rare congenital malformation. It has been mainly reported in the pediatric and neonatal population, with few reported cases in the adult population. In this case report, we discuss the presentation of an adult female patient with pituitary stalk duplication and primary empty sella (PES). A 46-year-old South Asian female presented with severe euvolemic hyponatremia. Initial investigation showed low serum osmolality, high urine osmolality, high urinary sodium concentration, and normal chest X-ray. On physical examination, the patient had underdeveloped secondary sexual characteristics. Laboratory tests showed low follicle-stimulating hormone, luteinizing hormone, and estradiol. Prolactin was moderately elevated, morning cortisol was low, adrenocorticotropic hormone (ACTH) was within the lower normal range, ACTH dynamic test was suboptimal, and insulin-like growth factor 1 was low. MRI showed empty sella with duplication of the pituitary stalk and third ventricle sagging. Pituitary stalk duplication is a rare congenital malformation that can be associated with other craniofacial abnormalities. Here, we describe the occurrence of pituitary stalk duplication with PES. It is not known if the two conditions are associated with each other. However, we speculate that the duplication of the stalk might have altered the normal anatomy of the aperture in the sellar diaphragm creating a space for the third ventricle to sag or herniate, as well as compressing the pituitary gland.
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spelling pubmed-105105662023-09-21 Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome Alkhyeli, Fatima Boharoon, Hessa Almarzouqi, Abdulla Cureus Endocrinology/Diabetes/Metabolism Pituitary duplication is a rare congenital malformation. It has been mainly reported in the pediatric and neonatal population, with few reported cases in the adult population. In this case report, we discuss the presentation of an adult female patient with pituitary stalk duplication and primary empty sella (PES). A 46-year-old South Asian female presented with severe euvolemic hyponatremia. Initial investigation showed low serum osmolality, high urine osmolality, high urinary sodium concentration, and normal chest X-ray. On physical examination, the patient had underdeveloped secondary sexual characteristics. Laboratory tests showed low follicle-stimulating hormone, luteinizing hormone, and estradiol. Prolactin was moderately elevated, morning cortisol was low, adrenocorticotropic hormone (ACTH) was within the lower normal range, ACTH dynamic test was suboptimal, and insulin-like growth factor 1 was low. MRI showed empty sella with duplication of the pituitary stalk and third ventricle sagging. Pituitary stalk duplication is a rare congenital malformation that can be associated with other craniofacial abnormalities. Here, we describe the occurrence of pituitary stalk duplication with PES. It is not known if the two conditions are associated with each other. However, we speculate that the duplication of the stalk might have altered the normal anatomy of the aperture in the sellar diaphragm creating a space for the third ventricle to sag or herniate, as well as compressing the pituitary gland. Cureus 2023-08-21 /pmc/articles/PMC10510566/ /pubmed/37736451 http://dx.doi.org/10.7759/cureus.43851 Text en Copyright © 2023, Alkhyeli et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Endocrinology/Diabetes/Metabolism
Alkhyeli, Fatima
Boharoon, Hessa
Almarzouqi, Abdulla
Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title_full Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title_fullStr Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title_full_unstemmed Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title_short Severe Hyponatremia in a 46-Year-Old Female With Pituitary Stalk Duplication and Primary Empty Sella Syndrome
title_sort severe hyponatremia in a 46-year-old female with pituitary stalk duplication and primary empty sella syndrome
topic Endocrinology/Diabetes/Metabolism
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10510566/
https://www.ncbi.nlm.nih.gov/pubmed/37736451
http://dx.doi.org/10.7759/cureus.43851
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