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Kałuża B, Furmanek M, Domański J, Żuk-Łapan A, Babula E, Poprawa I, Landowska M, Jarząbek K, Popczyńska J, Filipowicz P, Wielgolewska M, Walecki J, Franek E. The Influence of Pituitary Morphology on the Occurrence of Hormonal Disorders in Patients with Empty Sella or Partial Empty Sella. Biomedicines 2025; 13:762. [PMID: 40299333 PMCID: PMC12024816 DOI: 10.3390/biomedicines13040762] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2025] [Revised: 03/07/2025] [Accepted: 03/17/2025] [Indexed: 04/30/2025] Open
Abstract
Background/Objectives: The aim of the study was to prospectively assess the impact of certain parameters of pituitary morphology assessed with the use of magnetic resonance imaging on the occurrence of hormonal disorders in patients with primary partial empty sella (PES) or primary empty sella (ES). Methods: Forty-three patients were divided into two groups: group 1-patients with PES (n = 20); group 2-patients with ES (n = 23). Results: Patients with ES were characterized by larger both the transverse (14.8 ± 2.9 mm vs. 17.2 ± 2.9 mm, p = 0.016) and anteroposterior (AP) diameters of the pituitary (11.4 ± 1.4 mm vs. 13.2 ± 1.9 mm, p = 0.003), a smaller craniocaudal (CC) diameter (3.9 ± 0.62 mm vs. 2.2 ± 0.6 mm, p = 0.001), and a lower pituitary volume (332.8 ± 107.6 mm3 vs. 243.5 ± 70.9 mm3, p = 0.001). Moreover, an AP infundibular displacement was more common in patients with ES (7 [35%] vs. 16 [69.6%]., p = 0.023). Despite the fact that secondary adrenocortical insufficiency was shown to be significantly more common and ACTH levels to be significantly lower (27.5 ± 13.2 pg/mL vs. 21.8 ± 17.6 pg/mL, p = 0.039) in patients with ES (0 [0%] vs. 3 [13.4%], p = 0.046), univariate logistic regression did not reveal any significant associations of pituitary diameters, pituitary volume, or pituitary stalk displacement with endocrine disorders, such as secondary adrenocortical insufficiency or hyperprolactinemia, which was confirmed with multivariate logistic regression adjusted for age, sex, BMI, and arterial hypertension. Conclusions: Radiologically assessed CC, AP, and transverse pituitary diameters, pituitary volume, or pituitary stalk displacement in patients with PES or ES have no bearing on the rates of hormonal disorders. Nonetheless, certain hormonal disorders may be more common in patients with ES, which suggests a need for hormone-level assessments in this population.
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Affiliation(s)
- Bernadetta Kałuża
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Mariusz Furmanek
- Department of Radiology, National Institute of Medicine of the Ministry of the Interior and Administration, 02-507 Warsaw, Poland
- Department of Pediatric Radiology, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Jan Domański
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Aleksandra Żuk-Łapan
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Emilia Babula
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Iga Poprawa
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Małgorzata Landowska
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Karolina Jarząbek
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Justyna Popczyńska
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Paulina Filipowicz
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Małgorzata Wielgolewska
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Jerzy Walecki
- Department of Radiology, National Institute of Medicine of the Ministry of the Interior and Administration, 02-507 Warsaw, Poland
- Department of Radiology, Center of Postgraduate Medical Education, National Institute of Medicine of the Ministry of the Interior and Administration, 02-507 Warsaw, Poland
| | - Edward Franek
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Student Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Department of Human Epigenetics, Mossakowski Medical Research Centre Polish Academy of Sciences, 02-106 Warsaw, Poland
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Kałuża B, Furmanek M, Domański J, Żuk-Łapan A, Babula E, Poprawa I, Walecki J, Franek E. Relationship Between Radiological Features of Primary Empty or Primary Partial Empty Sella and Pituitary Hormone Levels. Biomedicines 2025; 13:722. [PMID: 40149698 PMCID: PMC11940325 DOI: 10.3390/biomedicines13030722] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2025] [Revised: 03/08/2025] [Accepted: 03/12/2025] [Indexed: 03/29/2025] Open
Abstract
Purpose: The purpose of this study was to assess the relationship between the radiological criteria determining an primary empty or primary partial empty sella and the pituitary hormone levels. Methods: Out of 594 patients who underwent pituitary magnetic resonance imaging (MRI), we selected 43 patients with primarily empty and partial empty sella and conducted a prospective evaluation of pituitary MRI in 2022. Pituitary craniocaudal (CC) diameter, pituitary volume, sellar volume, pituitary volume expressed as a percentage of sellar volume (bony sella), and pituitary height expressed as a percentage of sellar height (craniocaudal) were assessed. Serum pituitary hormone concentrations were measured, and a logistic regression analysis was performed to assess a relationship between the radiological and hormonal parameters. Results: Only six patients (14%) exhibited abnormal hormone levels. None of the assessed radiological parameters were correlated with the presence of the hormonal disorders either in the univariate or multivariate logistic regression analysis. The univariate logistic regression analysis revealed a significant relationship between age and the hormonal disorders (OR 0.916 [0.844-0.993]; p = 0.034), but this was not confirmed in the multivariate analysis. Conclusions: These findings suggest that radiological parameters alone are insufficient to predict hormonal dysfunction in patients with empty or partial empty sella. However, younger patients may be at a higher risk, warranting closer hormonal monitoring.
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Affiliation(s)
- Bernadetta Kałuża
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Mariusz Furmanek
- Department of Radiology, National Institute of Medicine of the Ministry of the Interior and Administration, 02-507 Warsaw, Poland
- Department of Pediatric Radiology, Medical University of Warsaw, 02-091 Warsaw, Poland
| | - Jan Domański
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Aleksandra Żuk-Łapan
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Emilia Babula
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Iga Poprawa
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
| | - Jerzy Walecki
- Department of Radiology, National Institute of Medicine of the Ministry of the Interior and Administration, 02-507 Warsaw, Poland
| | - Edward Franek
- Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland (E.B.)
- Students Scientific Group of the Medical University of Warsaw at the Department of Internal Medicine, Endocrinology and Diabetology, National Medical Institute of the Ministry of the Interior and Administration in Warsaw, 02-507 Warsaw, Poland
- Department of Human Epigenetics, Mossakowski Medical Research Centre, Polish Academy of Sciences, 02-106 Warsaw, Poland
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Padovano Sorrentino F, Chiloiro S, Giampietro A, Bianchi A, Pontecorvi A, De Marinis L. Empty sella syndrome: an update. Pituitary 2024; 28:13. [PMID: 39738761 DOI: 10.1007/s11102-024-01475-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/26/2024] [Indexed: 01/02/2025]
Abstract
INTRODUCTION Empty sella is characterized by a flattened profile of the pituitary gland that represents in most cases only a radiological incidental finding. When endocrine, ophthalmic, and neurological symptoms occur, this condition is described as empty sella syndrome. MATERIALS AND METHODS We searched MEDLINE (PubMed database) with the data filter 2024-2009 using the keywords listed above. The articles met the following inclusion criteria: 1) written in English; 2) published between 1 January 2009, and 31 December 2023; 3) original studies and case series on the epidemiology, characteristics, and treatment of empty sella and empty sella syndrome. Exclusion criteria included: articles written in languages other than English and single case report. RESULTS The incidence of empty sella is about 12%, when it comes to neuroimaging, while in clinical practice it can reach 35%. Endocrine pituitary disorders, defined as at least one hormone deficit, were reported in 19% to 40% of patients. Magnetic nuclear imaging (MRI) is confirmed to be the gold standard for radiological diagnosis of empty sella. If no alterations are detected it is suggested a careful revaluation at 24-36 months, in relation to the low risk of progression to PES syndrome. CONCLUSIONS Empty sella is, in most cases, a radiological finding without clinical manifestations. In people carrying empty sella hormonal pituitary evaluation should be assessed at the moment of the diagnosis, along with a careful imaging using MRI.
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Affiliation(s)
- Francesco Padovano Sorrentino
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
| | - Sabrina Chiloiro
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
| | - Antonella Giampietro
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
| | - Antonio Bianchi
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
| | - Alfredo Pontecorvi
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
| | - Laura De Marinis
- Dipartimento di Medicina Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy.
- Dipartimento di Endocrinologia, Diabetologia e Medicina Interna, Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy.
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Arkoudis NA, Davoutis E, Siderakis M, Papagiannopoulou G, Gouliopoulos N, Tsetsou I, Efthymiou E, Moschovaki-Zeiger O, Filippiadis D, Velonakis G. Idiopathic intracranial hypertension: Imaging and clinical fundamentals. World J Radiol 2024; 16:722-748. [PMID: 39801664 PMCID: PMC11718525 DOI: 10.4329/wjr.v16.i12.722] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2024] [Revised: 11/15/2024] [Accepted: 12/11/2024] [Indexed: 12/27/2024] Open
Abstract
Neuroimaging is a paramount element for the diagnosis of idiopathic intracranial hypertension, a condition characterized by signs and symptoms of raised intracranial pressure without the identification of a mass or hydrocephalus being recognized. The primary purpose of this review is to deliver an overview of the spectrum and the specific role of the various imaging findings associated with the condition while providing imaging examples and educational concepts. Clinical perspectives and insights into the disease, including treatment options, will also be discussed.
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Affiliation(s)
- Nikolaos-Achilleas Arkoudis
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Efstathia Davoutis
- School of Medicine, National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Manos Siderakis
- Department of Radiology, Agios Savas Anticancer Hospital, Athens 11522, Greece
| | - Georgia Papagiannopoulou
- 2nd Department of Neurology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Nikolaos Gouliopoulos
- 2nd Department of Ophthalmology, “Attikon” University Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Ilianna Tsetsou
- Department of Imaging and Interventional Radiology, “Sotiria” General and Chest Diseases Hospital of Athens, Athens 11527, Greece
| | - Evgenia Efthymiou
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Ornella Moschovaki-Zeiger
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Dimitrios Filippiadis
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Georgios Velonakis
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
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Ran C, Peng G, Shen R, Liao Q, Liao M, Wang Q, Zhou L, Zheng H, Long M. Efficacy of GnRH Pulses in Hypogonadism Secondary to Primary Empty Sella: Case Report. Reprod Sci 2024; 31:3892-3898. [PMID: 38958919 PMCID: PMC11612006 DOI: 10.1007/s43032-024-01637-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2024] [Accepted: 06/24/2024] [Indexed: 07/04/2024]
Abstract
This study aims to assess the effectiveness of pulsed gonadotropin-releasing hormone (GnRH) micropump replacement therapy in the treatment of hypogonadotropic hypogonadism (HH) caused by primary empty sella (PES).The efficacy of pulsed GnRH replacement therapy using the micropump was evaluated in a middle-aged male patient with HH who had experienced the loss of his only child. Relevant literature was also consulted to compare the differences between pulse GnRH treatment and conventional treatment in terms of the development of secondary sexual characteristics, sex hormone levels, sperm production rate, and sperm activity rate in male patient with HH.In this report, a 45-year-old male diagnosed with HH and PES presented with fatigue and decreased libido. The main characteristics included decreased follicle stimulating hormone (FSH) levels of 0.03 mIU/mL, luteinizing hormone (LH) levels of 0.02 mIU/mL, and testosterone (T) levels of 0.72 nmol/L. Magnetic resonance imaging (MRI) revealed an empty sella. Semen analysis showed a small number of normal sperm with reduced motility. During treatment with the micropump pulse GnRH, the patient experienced no side effects and showed improvements in fatigue, reduced libido, sexual urge, anxiety, and feelings of inferiority. LH, FSH, and T levels returned to normal, while sperm activity rate increased to 79.9%. Ultimately, the patient's spouse achieved a natural pregnancy.Pulsed gonadotropin delivery using the micropump demonstrates good efficacy and tolerability, and aligns more closely with the physiological rhythm of GnRH secretion in the human body.
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Affiliation(s)
- Chenxi Ran
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China
| | - Guiliang Peng
- Department of Endocrinology, The First Affiliated Hospital, Southwest Hospital, Army Medical University, No.30 Gaotanyan Zhengjie, Shapingba District, Chongqing, 400038, PR China
| | - Rufei Shen
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China
| | - Qian Liao
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China
| | - Mingyu Liao
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China
| | - Qixian Wang
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China
| | - Ling Zhou
- Department of Endocrinology, The First Affiliated Hospital, Southwest Hospital, Army Medical University, No.30 Gaotanyan Zhengjie, Shapingba District, Chongqing, 400038, PR China
| | - Hongting Zheng
- Department of Endocrinology, The Second Affiliated Hospital, Xinqiao Hospital, Army Medical University, No.83 Xinqiao Zhengjie, Shapingba District, Chongqing, 400037, PR China.
| | - Min Long
- Department of Endocrinology, The First Affiliated Hospital, Southwest Hospital, Army Medical University, No.30 Gaotanyan Zhengjie, Shapingba District, Chongqing, 400038, PR China.
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Iglesias P. An Update on Advances in Hypopituitarism: Etiology, Diagnosis, and Current Management. J Clin Med 2024; 13:6161. [PMID: 39458112 PMCID: PMC11508259 DOI: 10.3390/jcm13206161] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2024] [Revised: 10/06/2024] [Accepted: 10/09/2024] [Indexed: 10/28/2024] Open
Abstract
This article provides an updated review of hypopituitarism (HP), an endocrine disorder characterized by a deficiency of one or more pituitary hormones. The various etiologies are reviewed, including pituitary neuroendocrine tumors (PitNETs), hypothalamic lesions, genetic mutations, and acquired factors such as head trauma, medications, neoplasms, and infiltrative diseases. It is noted that PitNETs are responsible for approximately half of the cases in adults, whereas in children the causes are predominantly congenital. Diagnosis is based on clinical evaluation and hormonal testing, with identification of the specific hormonal deficiencies essential for effective treatment. Laboratory tests present challenges and limitations that must be understood and addressed. Hormone replacement therapy is the mainstay of treatment, significantly improving patients' quality of life. It is important to know the possible interactions between hormone replacement therapies in HP. Recent advances in understanding the pathophysiology of HP and the importance of a multidisciplinary approach to the management of associated complications are discussed. This article emphasizes the need for comprehensive evaluation and continuous follow-up to optimize outcomes in patients with HP and highlights the importance of ongoing research to improve diagnostic and treatment strategies.
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Affiliation(s)
- Pedro Iglesias
- Department of Endocrinology and Nutrition, Hospital Universitario Puerta de Hierro Majadahonda, Calle Joaquín Rodrigo, 1, 28222 Majadahonda, Madrid, Spain;
- Instituto de Investigación Sanitaria Puerta de Hierro Segovia de Arana, 28222 Majadahonda, Madrid, Spain
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Grandi G, Di Stefano M, Cebrelli C, Mengoli C, Di Sabatino A. Infections and gender: clues for diagnosis of adrenal insufficiency-a case report and a review of the literature. Intern Emerg Med 2024; 19:1821-1828. [PMID: 38888722 PMCID: PMC11466904 DOI: 10.1007/s11739-024-03613-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2024] [Accepted: 04/09/2024] [Indexed: 06/20/2024]
Abstract
The clinical presentation of adrenal insufficiency, a condition causing adrenal hormone deficiency, is characterised by non-specific symptoms and signs: consequently, an important diagnostic delay is often evident which correlates with an increased mortality. This case report shows how the clustering of some symptoms and signs may hamper the diagnostic suspicion for this condition: serum electrolyte alterations and weight loss, when associated to recurrent infections and, in female patients, an empty sella may further guide the clinician towards a diagnosis of adrenal insufficiency. Accordingly, a clinical approach taking into account gender medicine could improve the diagnostic workup.
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Affiliation(s)
- Giacomo Grandi
- Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia, P.le Camillo Golgi 2, 27100, Pavia, Italy
| | - Michele Di Stefano
- Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia, P.le Camillo Golgi 2, 27100, Pavia, Italy
| | - Chiara Cebrelli
- Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia, P.le Camillo Golgi 2, 27100, Pavia, Italy
| | - Caterina Mengoli
- Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia, P.le Camillo Golgi 2, 27100, Pavia, Italy
| | - Antonio Di Sabatino
- Department of Internal Medicine, IRCCS San Matteo Hospital Foundation, University of Pavia, P.le Camillo Golgi 2, 27100, Pavia, Italy.
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Castets S, Albarel F, Bachelot A, Brun G, Bouligand J, Briet C, Bui Quoc E, Cazabat L, Chabbert-Buffet N, Christin-Maitre S, Courtillot C, Cuny T, De Filippo G, Donadille B, Illouz F, Pellegrini I, Reznik Y, Saveanu A, Teissier N, Touraine P, Vantyghem MC, Vergier J, Léger J, Brue T, Reynaud R. Position statement on the diagnosis and management of congenital pituitary deficiency in adults: The French National Diagnosis and Treatment Protocol (NDTP). ANNALES D'ENDOCRINOLOGIE 2024; 85:327-339. [PMID: 38452869 DOI: 10.1016/j.ando.2024.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 03/09/2024]
Abstract
Pituitary deficiency, or hypopituitarism, is a rare chronic disease. It is defined by insufficient synthesis of one or more pituitary hormones (growth hormone, TSH, ACTH, LH-FSH, prolactin), whether or not associated with arginine vasopressin deficiency (formerly known as diabetes insipidus). In adult patients, it is usually acquired (notably during childhood), but can also be congenital, due to abnormal pituitary development. The present study focuses on congenital pituitary deficiency in adults, from diagnosis to follow-up, including special situations such as pregnancy or the elderly. The clinical presentation is highly variable, ranging from isolated deficit to multiple deficits, which may be part of a syndromic form or not. Diagnosis is based on a combination of clinical, biological (assessment of all hormonal axes), radiological (brain and hypothalamic-pituitary MRI) and genetic factors. Treatment consists in hormonal replacement therapy, adapted according to the period of life and the deficits, which may be progressive. Comorbidities, risk of complications and acute decompensation, and the impact on fertility and quality of life all require adaptative multidisciplinary care and long-term monitoring.
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Affiliation(s)
- Sarah Castets
- Service de pédiatrie multidisciplinaire, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Timone Enfants, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France.
| | - Frédérique Albarel
- Service d'endocrinologie, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Conception, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France
| | - Anne Bachelot
- IE3M, ICAN, Department of Endocrinology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Centre de Référence des Pathologies Gynécologiques Rares, hôpital Pitié-Salpêtrière, AP-HP, Paris, France; Sorbonne université, Paris, France
| | - Gilles Brun
- Aix-Marseille University, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Assistance Publique Hôpitaux de Marseille, Reference Center for Rare Pituitary Diseases HYPO, Assistance-Publique des Hôpitaux de Marseille, Laboratory of Molecular Biology, Conception Hospital, Marseille, France; Hôpital Européen, Pôle imagerie médicale, 13003, Marseille, France
| | - Jérôme Bouligand
- Molecular Genetic, Pharmacogenetic and Hormonology, Kremlin-Bicêtre Hospital, Paris-Saclay University, AP-HP, Le Kremlin-Bicêtre, France
| | - Claire Briet
- Département d'endocrinologie-diabétologie nutrition, Centre de référence des maladies rares de la Thyroïde et des Récepteurs Hormonaux, Endo-ERN centre for rare endocrine diseases, CHU d'Angers, 4, rue larrey, 49100 Angers, France; Laboratoire MITOVASC, UMR CNRS 6015, Inserm 1083, Université d'Angers, rue Roger Amsler, 49100 Angers, France
| | - Emmanuelle Bui Quoc
- Ophthalmology Department, Robert-Debré University Hospital, Assistance publique-Hôpitaux de Paris, Paris, France
| | - Laure Cazabat
- Department of Endocrinology, Diabetology and Nutrition, Ambroise Paré Hospital, AP-HP, UVSQ, Boulogne-Billancourt, France
| | - Nathalie Chabbert-Buffet
- Department of Gynecology and Obstetrics, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, 75020 Paris, France
| | - Sophie Christin-Maitre
- Department of Endocrinology, Diabetology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement (CMERC), Centre de Compétence HYPO, Hôpital Saint-Antoine, Sorbonne University, Assistance publique-Hôpitaux de Paris, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - Carine Courtillot
- IE3M, ICAN, Department of Endocrinology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Centre de Référence des Pathologies Gynécologiques Rares, hôpital Pitié-Salpêtrière, AP-HP, Paris, France
| | - Thomas Cuny
- Department of Endocrinology, Diabetology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement (CMERC), Centre de Compétence HYPO, Hôpital Saint-Antoine, Sorbonne University, Assistance publique-Hôpitaux de Paris, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - Gianpaolo De Filippo
- Service d'endocrinologie et diabétologie pédiatrique, centre de référence des maladies endocriniennes de la croissance et du développement, hôpital universitaire Robert-Debré, université Paris Cité, Assistance publique-Hôpitaux de Paris, Paris, France
| | - Bruno Donadille
- Department of Endocrinology, Diabetology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement (CMERC), Centre de Compétence HYPO, Hôpital Saint-Antoine, Sorbonne University, Assistance publique-Hôpitaux de Paris, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - Frédéric Illouz
- Département d'endocrinologie-diabétologie nutrition, Centre de référence des maladies rares de la Thyroïde et des Récepteurs Hormonaux, Endo-ERN centre for rare endocrine diseases, CHU d'Angers, 4, rue larrey, 49100 Angers, France; Laboratoire MITOVASC, UMR CNRS 6015, Inserm 1083, Université d'Angers, rue Roger Amsler, 49100 Angers, France
| | - Isabelle Pellegrini
- Service d'endocrinologie, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Conception, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France
| | - Yves Reznik
- Endocrinology and Diabetes Department, CHU Côte de Nacre and Unicaen, Caen Cedex, France
| | - Alexandru Saveanu
- Aix-Marseille University, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Assistance Publique Hôpitaux de Marseille, Reference Center for Rare Pituitary Diseases HYPO, Assistance-Publique des Hôpitaux de Marseille, Laboratory of Molecular Biology, Conception Hospital, Marseille, France
| | - Natacha Teissier
- Department of Pediatric Otolaryngology, Robert Debré Hospital, AP-HP Nord, Paris, France
| | - Philippe Touraine
- Service d'endocrinologie et médecine de la reproduction, centre de maladies endocrinennes rares de la croissance et du développement, médecine-hôpital Pitié-Salpêtrière, Sorbonne université, Paris, France
| | - Marie-Christine Vantyghem
- Service d'endocrinologie, diabétologie et maladies métaboliques, CHRU de Lille, rue Polonowski, Lille cedex, France
| | - Julia Vergier
- Service de pédiatrie multidisciplinaire, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Timone Enfants, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France
| | - Julianne Léger
- Service d'endocrinologie et diabétologie pédiatrique, centre de référence des maladies endocriniennes de la croissance et du développement, hôpital universitaire Robert-Debré, université Paris Cité, Assistance publique-Hôpitaux de Paris, Paris, France; Université Paris Cité, NeuroDiderot, Institut National de la Santé et de la Recherche Médicale (INSERM) UMR 1141, Paris, France
| | - Thierry Brue
- Service de pédiatrie multidisciplinaire, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Timone Enfants, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France; Aix-Marseille University, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Assistance Publique Hôpitaux de Marseille, Reference Center for Rare Pituitary Diseases HYPO, Assistance-Publique des Hôpitaux de Marseille, Laboratory of Molecular Biology, Conception Hospital, Marseille, France; Inserm, MMG, Laboratory of Molecular Biology, Hospital La Conception, Aix-Marseille University, AP-HM, Marseille, France
| | - Rachel Reynaud
- Service de pédiatrie multidisciplinaire, centre de référence des maladies rares de l'hypophyse HYPO, hôpital de la Timone Enfants, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Marseille, France; Aix-Marseille University, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Assistance Publique Hôpitaux de Marseille, Reference Center for Rare Pituitary Diseases HYPO, Assistance-Publique des Hôpitaux de Marseille, Laboratory of Molecular Biology, Conception Hospital, Marseille, France; Inserm, MMG, Laboratory of Molecular Biology, Hospital La Conception, Aix-Marseille University, AP-HM, Marseille, France
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9
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Lundholm MD, Yogi-Morren D. A Comprehensive Review of Empty Sella and Empty Sella Syndrome. Endocr Pract 2024; 30:497-502. [PMID: 38484938 DOI: 10.1016/j.eprac.2024.03.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2023] [Revised: 03/05/2024] [Accepted: 03/07/2024] [Indexed: 04/05/2024]
Abstract
OBJECTIVE Incidental radiographic findings of an empty sella are prevalent in up to 35% of the general population. While empty sella was initially considered clinically insignificant, a subset of patients exhibits endocrine or neuro-ophthalmologic manifestations which are diagnostic of empty sella syndrome (ESS). Recent studies suggest that more patients are affected by ESS than previously recognized, necessitating a deeper understanding of this condition. This comprehensive review describes a practical approach to evaluating and managing ESS. METHODS Literature review was conducted on etiologies and risk factors associated with primary and secondary empty sella, the radiologic features that differentiate empty sella from other sellar lesions, and the role of clinical history and hormone testing in identifying patients with ESS, as well as treatment modalities. RESULTS Pituitary function testing for somatotroph, lactotroph, gonadotroph, corticotroph, and thyrotroph abnormalities is necessary when suspecting ESS. While an isolated empty sella finding does not require treatment, ESS may require pharmacologic or surgical interventions to address hormone deficits or intracranial hypertension. Targeted hormone replacement as directed by the endocrinologist should align with guidelines and patient-specific needs. Treatment may involve a multidisciplinary collaboration with neurology, neurosurgery, or ophthalmology to address patient symptoms. CONCLUSION This review underscores the evolving understanding of ESS, stressing the significance of accurate diagnosis and tailored management to mitigate potential neurologic and endocrine complications in affected individuals.
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Affiliation(s)
- Michelle D Lundholm
- Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic, Cleveland, Ohio
| | - Divya Yogi-Morren
- Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic, Cleveland, Ohio.
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10
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Dapaah-Afriyie K. 1-Minute Pearls/Pitfalls for the Clinician. JOURNAL OF BROWN HOSPITAL MEDICINE 2024; 3:89973. [PMID: 40027393 PMCID: PMC11864449 DOI: 10.56305/001c.89973] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 10/16/2023] [Accepted: 11/08/2023] [Indexed: 03/05/2025]
Abstract
This article describes some pearls/pitfalls pertinent to empty sella and levothyroxine dose adjustment with certain medications/conditions.
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Affiliation(s)
- Kwame Dapaah-Afriyie
- Division of Hospital Medicine, Department of Medicine Miriam Hospital
- Department of Medicine Warren Alpert Medical School at Brown University
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11
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Rajesh M, Omer T, Chinniah S. Anterior hypopituitarism due to primary empty sella syndrome in a critically unwell patient. BMJ Case Rep 2023; 16:e255879. [PMID: 38081743 PMCID: PMC10728910 DOI: 10.1136/bcr-2023-255879] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2023] Open
Abstract
A previously fit and well male in his early 40s, presented to the emergency department, hypotensive, bradycardic and hypothermic with reduced levels of consciousness after being found diaphoretic and unwell at his home. Despite fluid resuscitation with warmed saline, he remained hypotensive and required vasopressor support leading to intensive care admission. Initially, the patient was managed for suspected meningoencephalitis but was later found to have hypopituitarism leading to secondary hypothyroidism and adrenal insufficiency. Subsequent investigations revealed it is due to empty sella syndrome.
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Affiliation(s)
- Mariya Rajesh
- Foundation School, Northampton General Hospital NHS Trust, Northampton, UK
| | - Tahir Omer
- Northampton General Hospital NHS Trust, Northampton, Northamptonshire, UK
| | - Sadasivan Chinniah
- Northampton General Hospital NHS Trust, Northampton, Northamptonshire, UK
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12
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Sugimoto R, Kenzaka T, Nishisaki H. Hyponatremia due to adrenal insufficiency after a Mamushi bite: a case report. BMC Endocr Disord 2023; 23:219. [PMID: 37821844 PMCID: PMC10566049 DOI: 10.1186/s12902-023-01466-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2023] [Accepted: 09/20/2023] [Indexed: 10/13/2023] Open
Abstract
BACKGROUND Mamushi bites are the most common venomous snake bites in Japan, with known complications including rhabdomyolysis and acute kidney injury; however, adrenal insufficiency as a result of snake bites has not been previously reported. We report a case of empty sella with transient adrenal insufficiency during hospitalization for a Mamushi bite. CASE PRESENTATION An 84-year-old man was admitted to our hospital with a Mamushi bite on the right fifth finger. Serum sodium (Na) level remained in the normal range. On the ninth day of admission, he developed hyponatremia, with a serum Na level of 114 mEq/L and serum cortisol level of 4.0 μg/dL (reference value 4.5-21.1 μg/dL). His serum Na level was restored within the normal range after administration of corticosteroids with 3% NaCl solution. Both rapid adrenocorticotrophin and corticotropin-releasing hormone loading tests showed low cortisol response. Based on the results of the hormone loading tests, a diagnosis of pituitary adrenal insufficiency was made. Contrast-enhanced pituitary magnetic resonance imaging (MRI) showed primary empty sella. After discontinuation of corticosteroids, the hyponatremia did not recur, and the patient was discharged on the 24th day of hospitalization. After discharge, the patient visited an outpatient clinic, but hyponatremia recurrence was not observed. CONCLUSIONS This is the first report of hyponatremia due to pituitary adrenal insufficiency during hospitalization for a Mamushi bite in a patient with empty sella. When hyponatremia occurs during hospitalization for a Mamushi bite, cortisol measurement, hormone loading test, and head MRI should be performed to search for pituitary lesions because of the possibility of adrenal insufficiency caused by snake venom.
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Affiliation(s)
- Ryu Sugimoto
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, 2002-7 Iso, Hikami-Cho, Tamba, 669-3495, Japan.
| | - Tsuneaki Kenzaka
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, 2002-7 Iso, Hikami-Cho, Tamba, 669-3495, Japan
- Division of Community Medicine and Career Development, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan
| | - Hogara Nishisaki
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, 2002-7 Iso, Hikami-Cho, Tamba, 669-3495, Japan
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13
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Tanaka Y, Amano T, Takahashi A, Nishimura H, Yamanaka H, Yoneoka Y, Tsuji S, Murakami T. Locally advanced endometrial cancer with multiple immune-related adverse events coinciding with the complete response to radiotherapy after immune checkpoint inhibitor therapy: A case report. Gynecol Oncol Rep 2023; 49:101265. [PMID: 37705723 PMCID: PMC10495623 DOI: 10.1016/j.gore.2023.101265] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2023] [Revised: 08/25/2023] [Accepted: 08/26/2023] [Indexed: 09/15/2023] Open
Abstract
We report a case of a 70-year-old female patient with locally advanced endometrial cancer with primary empty sella who developed multiple immune-related adverse events (irAEs), including hypopituitarism coinciding with the complete response to radiotherapy after receiving immune checkpoint inhibitors. A computed tomography scan acquired after a traffic accident led to the discovery of endometrial cancer that had invaded the vulva and primary empty sella. Following adriamycin and cisplatin, pembrolizumab was administered for three cycles. No irAEs were observed during treatment, but the tumor was progressive. The patient underwent radiotherapy for the residual tumor. Four months after the last dose of pembrolizumab, hypopituitarism caused secondary adrenal insufficiency, primary hypothyroidism, and pseudogout at the end of radiotherapy. The tumor later achieved a complete response. In conclusion, radiotherapy after immune checkpoint inhibitor (ICI) therapy is expected to have an antitumor effect by stimulating tumor-specific immunity. However, proper management of irAEs is necessary. Patients with primary empty sella may be prone to pituitary insufficiency induced by ICIs.
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Affiliation(s)
- Yuji Tanaka
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Tsukuru Amano
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Akimasa Takahashi
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Hiroki Nishimura
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Hiroyuki Yamanaka
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Yutaka Yoneoka
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Shunichiro Tsuji
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
| | - Takashi Murakami
- Department of Obstetrics and Gynecology, Shiga University of Medical Science, 520-2192 / Seta Tsukinowa-cho, Otsu, Shiga, JAPAN
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Ji W, Chen X. Hand-Schüller-Christian syndrome combined with empty sella syndrome: A case report and literature review. Medicine (Baltimore) 2023; 102:e33216. [PMID: 36897687 PMCID: PMC9997762 DOI: 10.1097/md.0000000000033216] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Accepted: 02/16/2023] [Indexed: 03/11/2023] Open
Abstract
RATIONAL Hand-Schüller-Christian syndrome (HCS) is a rare disease with little clinical awareness, but the condition is more dangerous, and it combines with empty sella syndrome (ESS) which is extremely rare. PRESENTATION A 26-year-old male patient who had proptosis, headaches, and diabetes insipidus for more than 10 years, and chronic cough and wheeze for 8 years presented to our hospital due to an abrupt onset of chest pain for 2 days. DIAGNOSIS Hand-Schüller-Christian syndrome is diagnosed based on the typical clinical manifestations of diabetes insipidus and bilateral proptosis, magnetic resonance imaging (MRI) pituitary imaging and pathology. Empty sella syndrome is diagnosed based on hormonal indicators, clinical manifestations and MRI pituitary scan results. Type 1 respiratory failure and severe pneumonia can be diagnosed based on the results of clinical examination, chest imaging (including chest x-ray and computed tomography), pathology and blood gas analysis. Left pneumothorax can be diagnosed with chest imaging. INTERVENTIONS "Meropenem and Cefdinir" were given for antimicrobrial coverage, "Desmopressin acetate" for anti-diuretic treatment, "Forcodine" for cough relief, "Ambroxol and acetylcysteine" for phlegm reduction, and continuous closed chest drainage was performed. OUTCOMES The patient discharged after cough, wheezing, headache and other symptoms improved, and vital signs were stable. The patient has been followed up once a month for 17 months ongoing after discharge. At present, symptoms such as cough, sputum, and wheezing have improved considerably, and the mMRC score of dyspnea is 2 points. The reexamination of the chest X-ray shows that the absorption of lung exudates is better than before, and there is no recurrence of pneumothorax. LESSONS Consider whether isolated diabetic insipidus is related to HSC, and if so, conduct an MRI, a biopsy, and other examinations as soon as possible.
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Affiliation(s)
- Wei Ji
- The Second Clinical College of Fujian Medical University, Quanzhou City, China
| | - Xiaoyang Chen
- Department of Respiratory and Critical Care Medicine, the Second Affiliated Hospital of Fujian Medical University, Quanzhou City, China
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Korsbæk JJ, Jensen RH, Høgedal L, Molander LD, Hagen SM, Beier D. Diagnosis of idiopathic intracranial hypertension: A proposal for evidence-based diagnostic criteria. Cephalalgia 2023; 43:3331024231152795. [PMID: 36786317 DOI: 10.1177/03331024231152795] [Citation(s) in RCA: 13] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/15/2023]
Abstract
BACKGROUND Based on expert opinion, abducens nerve palsy and a neuroimaging criterion (≥3 neuroimaging signs suggestive of elevated intracranial pressure) were added to the diagnostic criteria for idiopathic intracranial hypertension. Our objective was to validate this. METHODS This prospective study included patients with new-onset idiopathic intracranial hypertension for a standardized work-up: interview, neuro-ophthalmological exam, lumbar puncture, neuroimaging. Neuroimaging was evaluated by a blinded neuroradiologist. RESULTS We included 157 patients classified as idiopathic intracranial hypertension (56.7%), probable idiopathic intracranial hypertension (1.9%), idiopathic intracranial hypertension without papilledema (idiopathic intracranial hypertension-without papill edema; 0%), suggested idiopathic intracranial hypertension-without papill edema (4.5%), or non-idiopathic intracranial hypertension (36.9%). Moderate suprasellar herniation was more common in idiopathic intracranial hypertension than non-idiopathic intracranial hypertension (71.4% versus 47.4%, p < 0.01), as was perioptic nerve sheath distension (69.8% versus 29.3%, p < 0.001), flattening of the globe (67.1% versus 11.1%, p < 0.001) and transverse sinus stenosis (60.2% versus 18.9%, p < 0.001). Abducens nerve palsy was of no diagnostic significance. Sensitivity of ≥3 neuroimaging signs was 59.5% and specificity was 93.5%. CONCLUSION Moderate suprasellar herniation, distension of the perioptic nerve sheath, flattening of the globe and transverse sinus stenosis were associated with idiopathic intracranial hypertension. We propose that idiopathic intracranial hypertension can be defined by two out of three objective findings (papilledema, opening pressure ≥25 cm cerebrospinal fluid and ≥3 neuroimaging signs).
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Affiliation(s)
- Johanne Juhl Korsbæk
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Denmark
| | - Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Denmark
| | - Lisbeth Høgedal
- Department of Radiology, Odense University Hospital, Denmark
| | | | - Snorre Malm Hagen
- Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark
| | - Dagmar Beier
- Department of Neurology, Odense University Hospital, Denmark.,Department of Clinical Research, University of Southern Denmark, Odense, Denmark.,OPEN, Odense Patient data Explorative Network, Odense University Hospital, Denmark
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16
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Urhan E, Hacioglu A, Okcesiz I, Karaca Z, Kara CS, Unluhizarci K. The coexistence of newly diagnosed acromegaly with primary empty sella: More frequent than expected? Growth Horm IGF Res 2023; 68:101521. [PMID: 36427362 DOI: 10.1016/j.ghir.2022.101521] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2022] [Revised: 11/06/2022] [Accepted: 11/08/2022] [Indexed: 11/13/2022]
Abstract
OBJECTIVE We investigated the coexistence of newly diagnosed acromegaly with primary empty sella (ES), which is considered to be a rare association, and the impact of ES on the laboratory, radiological and prognostic status of acromegaly. DESIGN Acromegaly patients diagnosed and followed-up between 2012 and 2021 were included. Empty sella was defined as the pituitary gland and adenoma filling <50% of the sella turcica on preoperative T1 magnetic resonance imaging (MRI). RESULTS 102 acromegalic patients (45 male, 57 female, 45.5 ± 12.8 (range: 20-70 years) were included and data of a median 3 years (range: 0.5-9 years) were presented. ES was detected in 19 (18.6%) patients and 4 had complete and 15 had partial ES. Although not significant, adenoma size and residual adenoma on MRI on postoperative 3rd month, and disease remission at last control were lower in acromegaly with ES than in acromegaly without ES, while the rate of female gender and remission on postoperative 3rd month were higher. While preoperative serum prolactin and nadir GH responses to OGTT were significantly lower in patients with ES, there was no difference in terms of other pituitary hormones among both groups. CONCLUSION The present study revealed the coexistence of newly diagnosed acromegaly with primary ES at a rate of nearly 20% which is more frequent than expected and this association is not rare. The presence of ES was not associated with any preoperative/postoperative pituitary hormone levels and remission status, except lower preoperative prolactin and nadir GH responses to OGTT.
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Affiliation(s)
- Emre Urhan
- Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey
| | - Aysa Hacioglu
- Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey
| | - Izzet Okcesiz
- Department of Radiology, Erciyes University Medical School, Kayseri, Turkey
| | - Zuleyha Karaca
- Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey
| | - Canan Sehit Kara
- Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey
| | - Kursad Unluhizarci
- Department of Endocrinology, Erciyes University Medical School, Kayseri, Turkey.
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17
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Hulens M, Dankaerts W, Rasschaert R, Bruyninckx F, De Mulder P, Bervoets C. The Link Between Empty Sella Syndrome, Fibromyalgia, and Chronic Fatigue Syndrome: The Role of Increased Cerebrospinal Fluid Pressure. J Pain Res 2023; 16:205-219. [PMID: 36721849 PMCID: PMC9884441 DOI: 10.2147/jpr.s394321] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2022] [Accepted: 01/17/2023] [Indexed: 01/26/2023] Open
Abstract
The etiopathogenesis of fibromyalgia (FM) and chronic fatigue syndrome (CFS) is not yet elucidated. Hypothalamo-pituitary-adrenal (HPA) axis dysfunction is reflected in the hormonal disturbances found in FM and CFS. Some study groups have introduced a novel hypothesis that moderate or intermittent intracranial hypertension may be involved in the etiopathogenesis of FM and CFS. In these conditions, hormonal disturbances may be caused by the mechanical effect of increased cerebrospinal fluid pressure, which hampers blood flow in the pituitary gland. Severe intracranial pressure may compress the pituitary gland, resulting in primary empty sella (ES), potentially leading to pituitary hormone deficiencies. The aim of this narrative review was to explore whether similar hormonal changes and symptoms exist between primary ES and FM or CFS and to link them to cerebrospinal fluid pressure dysregulation. A thorough search of the PubMed and Web of Science databases and the reference lists of the included studies revealed that several clinical characteristics were more prevalent in primary ES, FM or CFS patients than in controls, including increased cerebrospinal fluid pressure, obesity, female sex, headaches and migraine, fatigue, visual disturbances (visual acuity and eye motility abnormalities), vestibulocochlear disturbances (vertigo and neurosensorial hearing loss), and bodily pain (radicular pain and small-fiber neuropathy). Furthermore, challenge tests of the pituitary gland showed similar abnormalities in all three conditions: blunted adrenocorticotropic hormone, cortisol, growth hormone, luteinizing hormone, and thyroid stimulating hormone responses and an increased prolactin response. The findings of this narrative review provide further support for the hypothesis that moderately or intermittently increased cerebrospinal fluid pressure is involved in the pathogenesis of FM and CFS and should stimulate further research into the etiopathogenesis of these conditions.
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Affiliation(s)
- Mieke Hulens
- Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium,Correspondence: Mieke Hulens, Department of Rehabilitation Sciences, KU Leuven, Overwegstraat 14, 3051 Sint-Joris-Weert, Leuven, Belgium, Tel +32 477 338003, Fax +32 16 329197, Email
| | - Wim Dankaerts
- Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium
| | | | - Frans Bruyninckx
- Department of Physical Medicine & Rehabilitation, University Hospitals of Leuven, Leuven, Belgium
| | - Peter De Mulder
- Department of Anesthesiology and Pain Therapy, Imelda Hospital, Bonheiden, Belgium
| | - Chris Bervoets
- Department of Neurosciences, KU Leuven, Leuven, Belgium,Department of Ophthalmology, University Hospitals of Leuven, Leuven, Belgium,Department Adult Psychiatry, University Psychiatric Center of KU Leuven, Leuven, Belgium
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18
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Lu M, Ye J, Gao F. Analysis of clinical features of primary empty sella. ANNALES D'ENDOCRINOLOGIE 2023; 84:249-253. [PMID: 36642370 DOI: 10.1016/j.ando.2023.01.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/08/2022] [Revised: 12/13/2022] [Accepted: 01/02/2023] [Indexed: 01/13/2023]
Abstract
OBJECTIVE A retrospective analysis of clinical data of 60 patients with primary empty sella (PES) was conducted to further improve the understanding of the disease. METHODS The clinical data of 60 patients diagnosed with PES admitted to the Department of Endocrinology of Wuhan Union Hospital for different reasons from January 2000 to September 2018 were analyzed. RESULTS The 60 cases comprised 22 (36.67%) male and 38 (63.33%) female patients, for a male-to-female ratio of 1:1.73. There was predominance of 50-59 year-olds and women with multiple pregnancies. Of the 60 patients, 41.67% showed fatigue, 26.67% dizziness and headache, 21.67% nausea and vomiting, 18.33% anorexia, and 6.67% visual impairment and other symptoms. Twenty-seven patients (45.0%) had normal pituitary function, and 33 (55.0%) had hypopituitarism. Complete PES was more prone to result in hypopituitarism than partial PES. Men with PES were more likely to have hypopituitarism than women. CONCLUSION The incidence of PES was significantly higher in women than in men; PES was common in middle-aged and older women with multiple pregnancies. Symptoms were diverse and clinical manifestations may lack specificity compared to other diseases and need to be differentiated. About half of PES patients may develop hypopituitarism. Therefore, for patients with empty sella detected on magnetic resonance imaging (MRI) with or without clinical symptoms, the pituitary function should be thoroughly and promptly evaluated.
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Affiliation(s)
- Mengyu Lu
- Department of Endocrinology, Wuhan Fourth Hospital, 430000 Wuhan, China
| | - Jianzhen Ye
- Department of Endocrinology, Huangzhou District People's Hospital, Huanggang, 438000 Hubei, China
| | - Feng Gao
- Department of Endocrinology, Wuhan Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 430022 Wuhan, China.
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19
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King HJ, Luther E, Morell AA, Ivan M, Komotar RJ. Management of a Growth Hormone-Secreting Pituitary Macroadenoma Associated With Idiopathic Intracranial Hypertension and an Empty Sella. Cureus 2023; 15:e34471. [PMID: 36874650 PMCID: PMC9982051 DOI: 10.7759/cureus.34471] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/31/2023] [Indexed: 02/04/2023] Open
Abstract
Idiopathic intracranial hypertension (IIH) is a -condition associated with elevated intracranial pressure (ICP) and frequently presents with headaches, papilledema, and visual loss. Rarely, IIH has been reported in association with acromegaly. Although removal of the tumor may reverse this process, elevated ICP, especially in the setting of an otherwise empty sella, may result in a cerebrospinal fluid (CSF) leak that is exceedingly difficult to manage. We present the first case of a patient with a functional pituitary adenoma causing acromegaly associated with IIH and an otherwise empty sella and discuss our management paradigm for this rare condition.
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Affiliation(s)
- Hunter J King
- Neurosurgery, Drexel University College of Medicine, Philadelphia, USA
| | - Evan Luther
- Neurological Surgery, University of Miami Miller School of Medicine, Miami, USA
| | - Alexis A Morell
- Neurological Surgery, University of Miami Miller School of Medicine, Miami, USA
| | - Michael Ivan
- Neurological Surgery, University of Miami Miller School of Medicine, Miami, USA
| | - Ricardo J Komotar
- Neurological Surgery, University of Miami Miller School of Medicine, Miami, USA
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20
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Das L, Dutta P. Approach to a case with an unusual cause of hypopituitarism. J Clin Endocrinol Metab 2022; 108:1488-1504. [PMID: 36573291 DOI: 10.1210/clinem/dgac747] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2022] [Indexed: 12/29/2022]
Abstract
Hypopituitarism refers to insufficiency of one or more hormones of the pituitary and can be due to myriad causes. The clinical and radiological spectrum of the condition is heterogeneous, based on the age, gender, clinical setting and/or other past medical history. Hypopituitarism includes central hypocortisolism, hypothyroidism, hypogonadism and growth hormone deficiency. Both hypo- and hyperprolactinemia can be associated with hypopituitarism, with low prolactin signifying more extensive pituitary damage. Posterior pituitary insufficiency (arginine vasopressin deficiency), occurs either in isolation or with anterior pituitary hormone deficiency. Clinical symptomatology of hypopituitarism is usually non-specific and insidious in onset and progression. Overall, the most common cause of hypopituitarism is a pituitary adenoma and/or its management (surgery, radiotherapy, pharmacotherapy or a combination of these). However, it is this subset of patients which is more likely to be identified and managed timely, possibly alleviating the premature mortality associated with hypopituitarism. What is more challenging is the recognition of hypopituitarism in less common settings, which may be either due to direct involvement of the pituitary (infection, traumatic brain injury, or infiltrative causes) or indirectly as a consequence of the primary process (thalassemia, vasculotoxic snakebite, subarachnoid hemorrhage). These entities are often under-recognised, and increased awareness can help in greater recognition of the problem burden. Further, pituitary insufficiency in most of these settings is dynamic, which may progress, or rarely, show recovery of function. This renders complexity to the problem, but makes it even more imperative to suspect, screen and appropriately manage patients with less common causes of hypopituitarism.
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Affiliation(s)
- Liza Das
- Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh
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21
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Lerner A, Sheikh-Bahaei N, Go JL. Utility of Neuroimaging in the Management of Chronic and Acute Headache. Otolaryngol Clin North Am 2022; 55:559-577. [PMID: 35490044 DOI: 10.1016/j.otc.2022.02.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Imaging plays an important role in identifying the cause of the much less common secondary headaches. Such headaches may be caused by a variety of pathologic conditions which can be categorized as intracranial and extracranial. Idiopathic intracranial hypertension imaging findings include "empty sella," orbital changes, and dural venous sinus narrowing. Intracranial hypotension (ICH) is frequently caused by CSF leaks. Imaging findings include loss of the CSF spaces, downward displacement of the brain, as well as dural thickening and enhancement. Severe cases of ICH may result in subdural hematomas. A variety of intracranial and skull base tumors may cause headaches due to dural involvement. Extracranial tumors and lesions that frequently present with headaches include a variety of sinonasal tumors as well as mucoceles. Neurovascular compression disorders causing headaches include trigeminal and glossopharyngeal neuralgia. Imaging findings include displacement and atrophy of the cranial nerve caused by an adjacent arterial or venous structure.
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Affiliation(s)
- Alexander Lerner
- Keck Medical Center of USCD, Department of Radiology, 1500 San Pablo Street, 2nd Floor, Imaging, Los Angeles, CA 90033, USA.
| | - Nasim Sheikh-Bahaei
- Keck Medical Center of USCD, Department of Radiology, 1500 San Pablo Street, 2nd Floor, Imaging, Los Angeles, CA 90033, USA
| | - John L Go
- Keck Medical Center of USCD, Department of Radiology, 1500 San Pablo Street, 2nd Floor, Imaging, Los Angeles, CA 90033, USA
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22
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Abstract
The epidemiology of male hypogonadism has been understudied. Of the known causes of endogenous androgen deficiency, only Klinefelter syndrome is common with a likely population prevalence of greater than 5:10,000 men (possibly as high as 10-25:10,000). Mild traumatic injury might also be a common cause of androgen deficiency (prevalence 5-10:10,000 men), but large, long-term studies must be completed to confirm this prevalence estimation that might be too high. The classic causes of male androgen deficiency-hyperprolactinemia, pituitary macroadenoma, endogenous Cushing syndrome, and iron overload syndrome-are rare (prevalence < 10,000 men).
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Affiliation(s)
- Arthi Thirumalai
- Department of Medicine, University of Washington School of Medicine, Box 356420, 1959 Northeast Pacific Avenue, Seattle, WA 98195, USA
| | - Bradley D Anawalt
- Department of Medicine, University of Washington School of Medicine, Box 356420, 1959 Northeast Pacific Avenue, Seattle, WA 98195, USA.
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23
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Carosi G, Brunetti A, Mangone A, Baldelli R, Tresoldi A, Del Sindaco G, Lavezzi E, Sala E, Mungari R, Fatti LM, Galazzi E, Ferrante E, Indirli R, Biamonte E, Arosio M, Cozzi R, Lania A, Mazziotti G, Mantovani G. A Multicenter Cohort Study in Patients With Primary Empty Sella: Hormonal and Neuroradiological Features Over a Long Follow-Up. Front Endocrinol (Lausanne) 2022; 13:925378. [PMID: 35813618 PMCID: PMC9259926 DOI: 10.3389/fendo.2022.925378] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/21/2022] [Accepted: 05/19/2022] [Indexed: 11/27/2022] Open
Abstract
OBJECTIVE primary empty sella (PES) represents a frequent finding, but data on hormonal alterations are heterogeneous, and its natural history is still unclear. Our aim was to evaluate the pituitary function of patients with PES over a long follow-up. DESIGN multicenter retrospective cohort study enrolling patients referred between 1984-2020 to five Pituitary Units, with neuroradiological confirmed PES and a complete hormonal assessment. METHODS we analyzed hormonal (including basal and dynamic evaluations), clinical and neuroradiological data collected at diagnosis and at the last visit (at least 6 months of follow-up). RESULTS we recruited 402 patients (females=63%, mean age=51.5 ± 16 years) with PES (partial, total, undefined in 66%, 13% and 21%, respectively). Hypopituitarism was present in 40.5% (hypogonadism=20.4%, hypoadrenalism=14.7%, growth hormone deficiency=14.7%, hypothyroidism=10.2%, diabetes insipidus=1.5%; multiple deficiencies=11.4%) and hypeprolactinemia in 6.5%. Interestingly, hormonal alterations were diagnosed in 29% of incidental PES. Hypopituitarism was associated with male sex (p=0.02), suspected endocrinopathy (p<0.001), traumatic brain injury (p=0.003) and not with age, BMI, number of pregnancies and neuroradiological grade. A longitudinal assessment was possible in 166/402 (median follow-up=58 months). In 5/166 (3%), new deficiencies occurred, whereas 14/166 (8.4%) showed a hormonal recovery. A progression from partial to total PES, which was found in 6/98 patients assessed with a second imaging, was the only parameter significantly related to the hormonal deterioration (p=0.006). CONCLUSIONS this is the largest cohort of patients with PES reported. Hypopituitarism is frequent (40%) but hormonal deterioration seems uncommon (3%). Patients need to be carefully evaluated at diagnosis, even if PES is incidentally discovered.
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Affiliation(s)
- Giulia Carosi
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- Sapienza University of Rome, Department of Experimental Medicine, Rome, Italy
| | - Alessandro Brunetti
- Humanitas University, Department of Biomedical Sciences, Pieve Emanuele, Italy
- IRCCS Humanitas Research Hospital, Endocrinology, Diabetology and Andrology Unit, Rozzano, Italy
| | - Alessandra Mangone
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- University of Milan, Department of Clinical Sciences and Community Health, Milan, Italy
| | - Roberto Baldelli
- A.O. San Camillo Forlanini, Endocrinology Unit, Department of Oncology and Medical Specialities, Rome, Italy
| | | | - Giulia Del Sindaco
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- University of Milan, Department of Clinical Sciences and Community Health, Milan, Italy
| | - Elisabetta Lavezzi
- IRCCS Humanitas Research Hospital, Endocrinology, Diabetology and Andrology Unit, Rozzano, Italy
| | - Elisa Sala
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
| | - Roberta Mungari
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
| | - Letizia Maria Fatti
- IRCCS Istituto Auxologico Italiano, Department of Endocrine and Metabolic Diseases, Milan, Italy
| | - Elena Galazzi
- IRCCS Istituto Auxologico Italiano, Department of Endocrine and Metabolic Diseases, Milan, Italy
| | - Emanuele Ferrante
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
| | - Rita Indirli
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- University of Milan, Department of Clinical Sciences and Community Health, Milan, Italy
| | - Emilia Biamonte
- IRCCS Humanitas Research Hospital, Endocrinology, Diabetology and Andrology Unit, Rozzano, Italy
| | - Maura Arosio
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- University of Milan, Department of Clinical Sciences and Community Health, Milan, Italy
| | - Renato Cozzi
- Niguarda Hospital, Division of Endocrinology, Milan, Italy
| | - Andrea Lania
- Humanitas University, Department of Biomedical Sciences, Pieve Emanuele, Italy
- IRCCS Humanitas Research Hospital, Endocrinology, Diabetology and Andrology Unit, Rozzano, Italy
- *Correspondence: Andrea Lania,
| | - Gherardo Mazziotti
- Humanitas University, Department of Biomedical Sciences, Pieve Emanuele, Italy
- IRCCS Humanitas Research Hospital, Endocrinology, Diabetology and Andrology Unit, Rozzano, Italy
| | - Giovanna Mantovani
- Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy
- University of Milan, Department of Clinical Sciences and Community Health, Milan, Italy
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Morgunova TB, Maloletkina ES, Amergulov II, Glinkina IV, Pavlova MG, Fadeyev VV. Panhypopituitarism In HIV Patient Receiving Antiretroviral Therapy. RUSSIAN OPEN MEDICAL JOURNAL 2021. [DOI: 10.15275/rusomj.2021.0402] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
The article is dedicated to a clinical case of the panhypopituitarism development in a patient with HIV infection receiving antiretroviral therapy. The number of HIV-infected patients worldwide has increased significantly over the past decades. The introduction of highly active antiretroviral therapy into practice has significantly improved the prognosis for these patients. At the same time, the use of this therapy type is associated with a number of complications – in particular, with the development of the immune restoration syndrome. In addition, cases of endocrine dysfunction are described in many publications. Timely diagnosis and treatment of endocrine disorders would help avoiding complications caused by excess or deficiency of hormones.
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Affiliation(s)
- Tatyana B. Morgunova
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | | | - Ilya I. Amergulov
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Irina V. Glinkina
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Maria G. Pavlova
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Valentin V. Fadeyev
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
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25
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Sharavii VB, Shnayder NA, Sharavii LK, Petrova MM, Nasyrova RF. Empty Sella Prevalence: Step By Step. RUSSIAN OPEN MEDICAL JOURNAL 2021. [DOI: 10.15275/rusomj.2021.0206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
An empty sella (ES), also known as an empty sella turcica (empty pituitary fossa) is defined as an intrasellar herniation of subarachnoid space within the sella which leads to flattening of the pituitary gland towards the posteroinferior wall. Purpose — to analyze studies of ES prevalence in the world. Methods — We carried out a systematic search in both Russian and English databases (E-library, PubMed, GoogleScholar, OxfordPress, ClinicalKeys) using keywords “empty sella”, “empty sella turcica”, “empty pituitary fossa”, “prevalence” and their combinations. The period of search: 2000-2020. It is also worth noting that earlier publications of historical interest were included in the review. Results — We have analyzed 23 studies. They can be roughly devided into two groups: the first group – screening studies of ES, the second group – targeted studies of ES.
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Affiliation(s)
- Victoria B. Sharavii
- I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Natalya A. Shnayder
- V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Saint-Petersburg; V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk, Russia
| | - Lada K. Sharavii
- Public healthcare institution of Republic of Tuva Republican children’s hospital, Kyzyl, Russia
| | - Marina M. Petrova
- V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk, Russia
| | - Regina F. Nasyrova
- V.M. Bekhterev National Medical Research Center for Psychiatry and Neurology, Saint-Petersburg, Russia; Kazan Federal University, Kazan, Russia
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26
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Himes BT, Bhargav AG, Brown DA, Kaufmann TJ, Bancos I, Van Gompel JJ. Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing's disease? A single-institution experience. Neurosurg Focus 2021; 48:E3. [PMID: 32480375 DOI: 10.3171/2020.3.focus2084] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2020] [Accepted: 03/03/2020] [Indexed: 11/06/2022]
Abstract
OBJECTIVE Cushing's disease arises from functioning adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing's disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing's disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing's disease. METHODS A single-institution database of Cushing's disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing's disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI. RESULTS Of the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61-6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002). CONCLUSIONS Empty sella syndrome is associated with an increased rate of MRI-negative Cushing's disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.
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Affiliation(s)
| | - Adip G Bhargav
- 2Mayo Clinic Alix School of Medicine, Rochester, Minnesota
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Chen BS, Meyer BI, Saindane AM, Bruce BB, Newman NJ, Biousse V. Prevalence of Incidentally Detected Signs of Intracranial Hypertension on Magnetic Resonance Imaging and Their Association With Papilledema. JAMA Neurol 2021; 78:718-725. [PMID: 33871552 PMCID: PMC8056310 DOI: 10.1001/jamaneurol.2021.0710] [Citation(s) in RCA: 39] [Impact Index Per Article: 9.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2020] [Accepted: 02/19/2021] [Indexed: 12/18/2022]
Abstract
Importance Magnetic resonance imaging (MRI) signs of intracranial hypertension (IH) are traditionally associated with idiopathic intracranial hypertension (IIH), but these signs are also detected among individuals with primary headaches and among asymptomatic individuals without papilledema. Objective To examine the prevalence of MRI signs of IH among consecutive outpatients undergoing brain MRI for any clinical indication and to explore their association with papilledema. Design, Setting, and Participants This prospective cross-sectional study of outpatients undergoing brain MRI at 1 outpatient imaging facility was conducted between August 1, 2019, and March 31, 2020, with ocular fundus photographs taken concurrently. Radiographic images from consecutive adult patients who were undergoing brain MRI and able to participate in fundus photography were analyzed for MRI signs of IH. A univariate analysis using either Fisher exact tests or t tests was performed. Main Outcomes and Measures Prevalence of MRI signs of IH and prevalence of papilledema detected on ocular fundus photographs. Radiographic signs of IH included empty sella, optic nerve head protrusion, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, enlarged Meckel caves, cephaloceles, cerebellar tonsillar descent, and bilateral transverse venous sinus stenosis. Results A total of 388 patients were screened for eligibility; of those, 92 patients were excluded (58 declined participation, 16 were unable to consent, 14 were unable to complete fundus photography, and 4 completed MRI and fundus photography twice, so their second set of findings was removed). Among the 296 patients included in the study, the median age was 49.5 years (interquartile range, 37.8-62.0 years), and 188 patients (63.5%) were female. The most common indication for MRI was surveillance of a brain neoplasm (82 patients [27.7%]). Investigations of headaches (26 patients [8.8%]) and disorders of intracranial pressure (4 patients [1.4%]) were uncommon. At least 1 radiographic sign of IH was present in 145 patients (49.0%). Among 296 total study patients, 98 patients (33.1%) had empty sella, 47 patients (15.9%) had enlarged Meckel caves, 32 patients (10.8%) had increased perioptic cerebrospinal fluid, 23 patients (7.8%) had optic nerve tortuosity, 2 patients (0.7%) had scleral flattening, and 4 patients (1.4%) had cephaloceles. Bilateral transverse venous sinus stenosis was present in 6 of 198 patients (3.0%). Five patients (1.7%) had papilledema. Compared with patients without papilledema, those with papilledema had a significantly higher body mass index and history of IIH, in addition to an increased prevalence of empty sella, optic nerve tortuosity, and transverse venous sinus stenosis detected on MRI. The prevalence of papilledema increased from 2.8% among patients with at least 1 MRI sign of IH to 40.0% among patients with 4 or more MRI signs of IH. Conclusions and Relevance Magnetic resonance imaging signs of IH were common among patients undergoing brain MRI in this study but rarely associated with papilledema. The management of patients with incidentally detected signs of IH likely does not require systematic lumbar puncture unless concerning symptoms or papilledema are present.
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Affiliation(s)
- Benson S. Chen
- Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
| | - Benjamin I. Meyer
- Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
| | - Amit M. Saindane
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, Georgia
| | - Beau B. Bruce
- Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
- Department of Epidemiology, Emory University School of Medicine, Atlanta, Georgia
| | - Nancy J. Newman
- Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
- Department of Neurology, Emory University School of Medicine, Atlanta, Georgia
- Department of Neurological Surgery, Emory University School of Medicine, Atlanta, Georgia
| | - Valérie Biousse
- Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
- Department of Neurology, Emory University School of Medicine, Atlanta, Georgia
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28
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Li X, Chen L, Xu J, Xiong X, Deng Y. A case of cutis verticis gyrata related to pregnancy. Indian J Dermatol Venereol Leprol 2021; 87:533-534. [PMID: 33969669 DOI: 10.25259/ijdvl_751_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2020] [Accepted: 02/01/2021] [Indexed: 11/04/2022]
Affiliation(s)
- Xulin Li
- Department of Dermatology, Affifiliated Hospital of Southwest Medical University, Luzhou, Sichuan, China
| | - Lingna Chen
- Department of Dermatology, Affifiliated Hospital of Southwest Medical University, Luzhou, Sichuan, China
| | - Jixiang Xu
- Department of Dermatology, Affifiliated Hospital of Southwest Medical University, Luzhou, Sichuan, China
| | - Xia Xiong
- Department of Dermatology, Affifiliated Hospital of Southwest Medical University, Luzhou, Sichuan, China
| | - Yongqiong Deng
- Department of Dermatology, Affifiliated Hospital of Southwest Medical University, Luzhou, Sichuan, China
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Primary empty sella: The risk factors and associations with the cerebral small vessel diseases-An observational study. Clin Neurol Neurosurg 2021; 203:106586. [PMID: 33730618 DOI: 10.1016/j.clineuro.2021.106586] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2020] [Revised: 02/11/2021] [Accepted: 02/27/2021] [Indexed: 11/20/2022]
Abstract
OBJECTIVE To investigate the risk factors of primary empty sella (PES) and its associations with cerebral small vessel diseases (CSVD). METHODS A total of 132 consecutive patients were recruited from Department of Neurology, The Second Affiliated Hospital of Chongqing Medical University from December 2018 to January 2020, including 69 cases of PES, and age, gender-matched 63 subjects without PES. Demographics and clinical characteristics were recorded. Enlarged perivascular spaces (PVS) and white matter hyperintensities (WMH), which are image markers for CSVD, were assessed. Univariate logistic regression models and multivariate logistic regression models were performed to predict the independent risk factors of PES. RESULTS There was a significant difference in baseline characteristics in terms of hypertension (p < 0.001) and pregnancy (p = 0.019) between PES and the control group; among markers of CSVD, whole WMH (p = 0.030) and periventricular hyperintensities (PVH) (p = 0.027) were significantly different; however, no significant differences concerning deep WMH, total PVS, basilar ganglia-PVS and centrum semiovale-PVS (p > 0.05). After adjusting relevant potential confounders, multivariate logistic regression revealed hypertension (OR=3.158, 95 %CI: 1.452∼6.865, p = 0.004) and pregnancy (OR=2.236, 95 %CI: 1.036-4.826, p = 0.040) were independent risk factors for PES. CONCLUSION Hypertension and pregnancy are independent risk factors of PES. There is a possible correlation between PES and WMH, especially PVH, however, further studies are required to confirm these findings.
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Prodam F, Caputo M, Mele C, Marzullo P, Aimaretti G. Insights into non-classic and emerging causes of hypopituitarism. Nat Rev Endocrinol 2021; 17:114-129. [PMID: 33247226 DOI: 10.1038/s41574-020-00437-2] [Citation(s) in RCA: 33] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/19/2020] [Indexed: 12/11/2022]
Abstract
Hypopituitarism is defined as one or more partial or complete pituitary hormone deficiencies, which are related to the anterior and/or posterior gland and can have an onset in childhood or adulthood. The most common aetiology is a sellar or suprasellar lesion, often an adenoma, which causes hypopituitarism due to tumour mass effects, or the effects of surgery and/or radiation therapy. However, other clinical conditions, such as traumatic brain injury, and autoimmune and inflammatory diseases, can result in hypopituitarism, and there are also genetic causes of hypopituitarism. Furthermore, the use of immune checkpoint inhibitors to treat cancer is increasing the risk of hypopituitarism, with a pattern of hormone defects that is different from the classic patterns and depends on mechanisms that are specific for each drug. Moreover, autoantibody production against the pituitary and hypothalamus has been demonstrated in studies investigating the development or worsening of some cases of hypopituitarism. Finally, evidence suggests that posterior pituitary damage can affect oxytocin secretion. The aim of this Review is to summarize current knowledge on non-classic and emerging causes of hypopituitarism, so as to help clinicians improve early identification, avoid life-threatening events and improve the clinical care and quality of life of patients at risk of hypopituitarism.
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Affiliation(s)
- Flavia Prodam
- Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy
- Department of Health Sciences, Università del Piemonte Orientale, Novara, Italy
| | - Marina Caputo
- Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy
- Department of Health Sciences, Università del Piemonte Orientale, Novara, Italy
| | - Chiara Mele
- Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy
| | - Paolo Marzullo
- Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy
- Division of General Medicine, I.R.C.C.S. Istituto Auxologico Italiano, Ospedale San Giuseppe, Verbania, Italy
| | - Gianluca Aimaretti
- Endocrinology, Department of Translational Medicine, Università del Piemonte Orientale, Novara, Italy.
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Abstract
PURPOSE OF REVIEW Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure. RECENT FINDINGS MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential. SUMMARY MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.
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Affiliation(s)
| | - Lakshmi Leishangthem
- Department of Neurology and Ophthalmology, University of Connecticut, Farmington, Connecticut
| | - Heather E Moss
- Departments of Ophthalmology, Neurology and Neurosciences, Stanford University, Palo Alto, California, USA
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Grangeon L, O'Connor E, Danno D, Ngoc TMP, Cheema S, Tronvik E, Davagnanam I, Matharu M. Is pituitary MRI screening necessary in cluster headache? Cephalalgia 2021; 41:779-788. [PMID: 33406848 PMCID: PMC8166405 DOI: 10.1177/0333102420983303] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Objective To determine the prevalence and clinical predictors of pituitary adenomas in cluster headache patients, in order to determine the necessity of performing dedicated pituitary magnetic resonance imaging in patients with cluster headache. Methods A retrospective study was conducted of all consecutive patients diagnosed with cluster headache and with available brain magnetic resonance imaging between 2007 and 2017 in a tertiary headache center. Data including demographics, attack characteristics, response to treatments, results of neuroimaging, and routine pituitary function tests were recorded. Results Seven hundred and eighteen cluster headache patients attended the headache clinic; 643 underwent a standard magnetic resonance imaging scan, of whom 376 also underwent dedicated pituitary magnetic resonance imaging. Pituitary adenomas occurred in 17 of 376 patients (4.52%). Non-functioning microadenomas (n = 14) were the most common abnormality reported. Two patients, one of whom lacked the symptoms of pituitary disease, required treatment for their pituitary lesion. No clinical predictors of those adenomas were identified after multivariate analysis using random forests. Systematic pituitary magnetic resonance imaging scanning did not benefit even a single patient in the entire cohort. Conclusion The prevalence of pituitary adenomas in cluster headache is similar to that reported in the general population, thereby precluding an over-representation of pituitary lesions in cluster headache. We conclude that the diagnostic assessment of cluster headache patients should not include specific pituitary screening. Only patients with standard brain magnetic resonance imaging findings or symptoms suggestive of a pituitary disorder require brain magnetic resonance imaging with dedicated pituitary views.
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Affiliation(s)
- Lou Grangeon
- Headache and Facial Pain Group, UCL Queen Square Institute of Neurology, Queen Square, London, UK.,Headache and Facial Pain Group, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
| | - Emer O'Connor
- Headache and Facial Pain Group, UCL Queen Square Institute of Neurology, Queen Square, London, UK
| | - Daisuke Danno
- Headache and Facial Pain Group, UCL Queen Square Institute of Neurology, Queen Square, London, UK
| | | | - Sanjay Cheema
- Headache and Facial Pain Group, UCL Queen Square Institute of Neurology, Queen Square, London, UK
| | - Erling Tronvik
- Mathematics Institute of Orsay, Paris Sud University, Orsay, France.,Department of Neurology, St Olav's University Hospital, Trondheim, Norway.,NTNU (University of Science and Technology), Department of Neuromedicine and Movement Science, Trondheim, Norway
| | | | - Manjit Matharu
- Headache and Facial Pain Group, UCL Queen Square Institute of Neurology, Queen Square, London, UK
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Akkus G, Sözütok S, Odabaş F, Onan B, Evran M, Karagun B, Sert M, Tetiker T. Pituitary Volume in Patients with Primary Empty Sella and Clinical Relevance to Pituitary Hormone Secretion: A Retrospective Single Center Study. Curr Med Imaging 2021; 17:1018-1024. [PMID: 34036923 PMCID: PMC8653417 DOI: 10.2174/1573405617666210525111218] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2020] [Revised: 02/16/2021] [Accepted: 02/23/2021] [Indexed: 11/22/2022]
Abstract
BACKGROUND According to neuroradiological findings, empty sella seems to be deprived of pituitary tissue in sella turcica. Changing size of the pituitary volume is closely related to the occurrence of primary empty sella. The aim of the study is to determine pituitary dysfunction in patients with partial or total primary empty sella and the significance of pituitary volume measurements in these patients. METHODS This study was designed retrospectively. 67 patients (55 females, 12 males) diagnosed with primary empty sella syndrome between the years of 2015-2019 were included in the study. Patients were divided into two groups: partial (PES) and total (TES) empty sella by magnetic resonance imaging (MRI). Basal anterior pituitary and its hormones were assessed. We also included 26 healthy control subjects (19 females, 7 males) to compare the differences in pituitary volumes. Volumes were measured by using Osirix Dicom Viewer (Pixmeo SARL, Geneve, Swiss) in 3.0 Tesla scanner MRI. RESULTS 82.1% (n=55) of all patients were PES and the others were (n=12) TES. Hypopituitarism, known as one or more pituitary hormones deficiency, was found in 12 patients (17.9%). While 9 of them had total PES, the others had partial PES. Secondary adrenal insufficiency and gonadotropin deficiency were more prevalent in patients with TES. Mean volume measurements of patients with TES, PES and healthy subjects were 0.23±0.17, 0.35±0.15, 0.54±0.17 cm3, respectively. Except for IGF1 values (p=0.026), there was not any significant correlation found between the anterior pituitary hormones and volume measurements. CONCLUSION Although volume measurement has helped in the diagnosis of pituitary empty sella (partial or total), it does not seem to have any significant correlation with pituitary secretory function.
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Affiliation(s)
- Gamze Akkus
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
| | - Sinan Sözütok
- Faculty of Medicine, Division of Radiology, Cukurova University, , Adana, Turkey
| | - Fulya Odabaş
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
| | - Bilen Onan
- Faculty of Medicine, Division of Radiology, Cukurova University, Adana, Turkey
| | - Mehtap Evran
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
| | - Barış Karagun
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
| | - Murat Sert
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
| | - Tamer Tetiker
- Faculty of Medicine, Division of Endocrinology, Cukurova University, Adana, Turkey
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Perosevic M, Jones PS, Tritos NA. Magnetic resonance imaging of the hypothalamo-pituitary region. HANDBOOK OF CLINICAL NEUROLOGY 2021; 179:95-112. [PMID: 34225987 DOI: 10.1016/b978-0-12-819975-6.00004-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
The diagnosis and management of mass lesions in the sellar and parasellar areas remain challenging. When approaching patients with possible sellar or hypothalamic masses, it is important not only to focus on imaging but also detect possible pituitary hormone deficits or excess, in order to establish an appropriate diagnosis and initiate treatment. The imaging modalities used to characterize hypothalamic and pituitary lesions have significantly evolved over the course of the past several years. Computed tomography (CT) and CT angiography play a major role in detecting various sellar lesions, especially in patients who have contraindications to magnetic resonance imaging (MRI) and can also yield important information for surgical planning. However, MRI has become the gold standard for the detection and characterization of hypothalamic and pituitary tumors, infections, cystic, or vascular lesions. Indeed, the imaging characteristics of hypothalamic and sellar lesions can help narrow down the differential diagnosis preoperatively. In addition, MRI can help establish the relationship of mass lesions to surrounding structures. A pituitary MRI examination should be obtained if there is concern for mass effect (including visual loss, ophthalmoplegia, headache) or if there is clinical suspicion and laboratory evidence of either hypopituitarism or pituitary hormone excess. The information obtained from MRI images also provides us with assistance in planning surgery. Using intraoperative MRI can be very helpful in assessing the adequacy of tumor resection. In addition, MRI images yield reliable data that allow for noninvasive monitoring of patients postoperatively.
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Affiliation(s)
- Milica Perosevic
- Neuroendocrine Unit, Massachusetts General Hospital, Boston, MA, United States; Harvard Medical School, Boston, MA, United States.
| | - Pamela S Jones
- Department of Neurosurgery, Massachusetts General Hospital, Boston, MA, United States; Harvard Medical School, Boston, MA, United States
| | - Nicholas A Tritos
- Neuroendocrine Unit, Massachusetts General Hospital, Boston, MA, United States; Harvard Medical School, Boston, MA, United States
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Chiloiro S, Giampietro A, Bianchi A, De Marinis L. Empty sella syndrome: Multiple endocrine disorders. HANDBOOK OF CLINICAL NEUROLOGY 2021; 181:29-40. [PMID: 34238465 DOI: 10.1016/b978-0-12-820683-6.00003-8] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Empty sella is a pituitary disorder characterized by the herniation of the subarachnoid space within the sella turcica. This is often associated with a variable degree of flattening of the pituitary gland. Empty sella has to be distinguished in primary and secondary forms. Primary empty sella (PES) excludes any history of previous pituitary pathologies such as previous surgical, pharmacologic, or radiotherapy treatment of the sellar region. PES is considered an idiopathic disease and may be associated with idiopathic intracranial hypertension. Secondary empty sella, however, may occur after the treatment of pituitary tumors through neurosurgery or drugs or radiotherapy, after spontaneous necrosis (ischemia or hemorrhage) of chiefly adenomas, after pituitary infectious processes, pituitary autoimmune diseases, or brain trauma. Empty sella, in the majority of cases, is only a neuroradiological finding, without any clinical implication. However, empty sella syndrome is defined in the presence of pituitary hormonal dysfunction (more frequently hypopituitarism) and/or neurological symptoms due to the possible coexisting of idiopathic intracranial hypertension. Empty sella syndrome represents a peculiar clinical entity, characterized by heterogeneity both in clinical manifestations and in hormonal alterations, sometimes reaching severe extremes. For a proper diagnosis, management, and follow-up of empty sella syndrome, a multidisciplinary approach with the integration of endocrine, neurological, and ophthalmological experts is strongly advocated.
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Affiliation(s)
- Sabrina Chiloiro
- Pituitary Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Antonella Giampietro
- Pituitary Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Antonio Bianchi
- Pituitary Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Laura De Marinis
- Pituitary Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
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36
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Mehla S, Chua AL, Grosberg B, Evans RW. Primary Empty Sella. Headache 2020; 60:2522-2525. [PMID: 33038281 DOI: 10.1111/head.13987] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2020] [Accepted: 09/14/2020] [Indexed: 11/29/2022]
Affiliation(s)
- Sandhya Mehla
- Hartford Healthcare Headache Center University of Connecticut School of Medicine West Hartford CT USA
| | - Abigail L. Chua
- Hartford Healthcare Headache Center University of Connecticut School of Medicine West Hartford CT USA
| | - Brian Grosberg
- Hartford Healthcare Headache Center University of Connecticut School of Medicine West Hartford CT USA
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Ekhzaimy AA, Mujammami M, Tharkar S, Alansary MA, Al Otaibi D. Clinical presentation, evaluation and case management of primary empty sella syndrome: a retrospective analysis of 10-year single-center patient data. BMC Endocr Disord 2020; 20:142. [PMID: 32943019 PMCID: PMC7495892 DOI: 10.1186/s12902-020-00621-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2019] [Accepted: 09/06/2020] [Indexed: 11/24/2022] Open
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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Affiliation(s)
- Aishah A. Ekhzaimy
- Endocrinology and Diabetes Unit, Department of Medicine, College of Medicine and King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Muhammad Mujammami
- Endocrinology and Diabetes Unit, Department of Medicine, College of Medicine and King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Shabana Tharkar
- Prince Sattam Chair for Epidemiology and Public Health Research, Department of Family and Community Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Manahel A. Alansary
- Endocrinology and Diabetes Unit, Department of Medicine, College of Medicine and King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Daad Al Otaibi
- Endocrinology and Diabetes Unit, Department of Medicine, College of Medicine and King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
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Beysel S, Çalişkan M, Kizilgül M, Kan S, Özbek M, Çakal E. Clinical evaluation of pituitary insufficiency in adult population. Turk J Med Sci 2020; 50:917-921. [PMID: 32283895 PMCID: PMC7379402 DOI: 10.3906/sag-1908-19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2019] [Accepted: 04/11/2020] [Indexed: 11/03/2022] Open
Abstract
Background/aim This retrospective study aimed to investigate the clinical profile of pituitary insufficiency (PI) in adult population. Materials and methods One hundred and fifty patients who were diagnosed as having PI between 2012 and 2018 (53.3% female, mean age 48.13 ± 15.83 years) were retrospectively analyzed. Results Age at diagnosis was higher in females as compared with males (51.13 ± 15.95 vs. 44.70 ± 15.08 years, P = 0.012). The most frequent presenting signs were headache (29.4%) and visual disturbance (19.6%) in general. Females frequently presented with headache (33.3%), whereas males presented with sexual dysfunction (34.4%). The most frequent cause of PI was nonfunctional pituitary adenoma (28.8%) in general population. A frequent cause of PI was Sheehan’s syndrome (33.8%) among females and nonfunctional pituitary adenoma (38.6%) among males. Pituitary macroadenoma (75.8%) was frequent in pituitary tumors with PI. 55.3 % of the patients had 4 pituitary hormones deficiencies and 26.0% of patients had 3 pituitary hormones deficiencies. Gonadotropin deficiency was the leading pituitary hormone deficiency. The frequency of posttraumatic PI was 4.7% in the general population. Conclusion Nonfunctional pituitary adenoma was the most common cause of PI among males and Sheehan’s syndrome was a major etiologic factor in females. Sheehan’s syndrome remains an important health problem in Turkey although obstetric care has improved. Posttraumatic PI should be considered in the differential diagnosis of idiopathic PI.
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Affiliation(s)
- Selvihan Beysel
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
| | - Mustafa Çalişkan
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
| | - Muhammed Kizilgül
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
| | - Seyfullah Kan
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
| | - Mustafa Özbek
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
| | - Erman Çakal
- Department of Endocrinology and Metabolism, University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
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Magnetic Resonance Imaging of Idiopathic Intracranial Hypertension: Before and After Treatment. J Neuroophthalmol 2020; 39:324-329. [PMID: 31430269 DOI: 10.1097/wno.0000000000000792] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND This study aimed to identify the reversibility of MRI findings indicative of increased intracranial hypertension in idiopathic intracranial hypertension (IIH) patients after treatment. METHODS This retrospective, observational study included demographic and clinical data from 10 patients with IIH and 10 controls. Brain MRI findings in IIH patients were recorded twice: once when patients had papilledema and again after resolution of papilledema. Neuroradiologists graded MRI findings in both groups based on an imaging grading scale. RESULTS After resolution of papilledema, all patients showed improvement in 2 or more of the MRI characteristics of IIH. This was especially the case for the height of the midsagittal pituitary gland and optic nerve sheath thickness (ONST), which were significantly different in all pairwise group comparisons. Sellar configuration, globe configuration, and horizontal orbital optic nerve tortuosity were different between the IIH pre-treatment group and controls, but not between controls and the IIH post-treatment group. We found no difference in optic nerve head hyperintensity or optic nerve thickness among the 3 groups. CONCLUSIONS We demonstrated that several morphometric MRI characteristics in IIH are reversible to a certain extent after treatment. Enlarged subarachnoid spaces filled with cerebrospinal fluid seem to remain reduced, and the ONST and height of the pituitary gland are not fully normalized after treatment.
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Braiteh N, Breiteh N, Kashou HE. Sinus Bradycardia as a Rare and Unusual Presentation of Partial Empty Sella Syndrome: A Case Report. AMERICAN JOURNAL OF CASE REPORTS 2019; 20:1071-1074. [PMID: 31327866 PMCID: PMC6698065 DOI: 10.12659/ajcr.916812] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
BACKGROUND Empty sella syndrome (ESS) occurs when the pituitary gland becomes flattened or shrinks, which leads to filling of the sella turcica with cerebrospinal fluid. This causes the appearance of empty sella on imaging. ESS is often diagnosed after the workup of pituitary disorders, or as an incidental finding after brain imaging. To the best of our knowledge, this is the first case report in which ESS was diagnosed after an incidental finding of bradycardia pre-operatively. CASE REPORT We present the case of a 66-year-old man who was admitted electively to undergo a transforaminal lumbar interbody fusion at the level of L5-S1. He was found to be bradycardic pre-operatively. Upon further workup, he was found to have a thyroid-stimulating hormone (TSH) level of 0.358 uIU/ML (normal range, 0.465-4.680 uIU/ML) and a free thyroxine (FT4) level of 0.8 ng/dL (normal range, 0.8-2.2ng/dL). He also had a history of hypotestosteronemia. He was diagnosed with central hypothyroidism at the time of admission. Subsequently, a magnetic resonance imaging (MRI) scan of the brain was performed, and partial empty sella syndrome was diagnosed. CONCLUSIONS Bradycardia in the setting of empty sella syndrome is a rare condition. It is of utmost importance for clinicians to keep in mind a wide differential that includes other non-cardiac causes for common cardiac symptoms such as bradycardia.
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Affiliation(s)
- Nabil Braiteh
- Department of Cardiology, United Health Services Hospitals, Wilson Medical Center, Johnson City, NY, USA
| | - Nour Breiteh
- Center for Public Health Practice at the Faculty of Health Sciences, American University of Beirut, Beirut, Lebanon
| | - Hisham E Kashou
- Department of Cardiology, United Health Services Hospitals, Wilson Medical Center, Johnson City, NY, USA
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Guinto G, Nettel B, Hernández E, Gallardo D, Aréchiga N, Mercado M. Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome. World Neurosurg 2019; 126:e953-e958. [DOI: 10.1016/j.wneu.2019.02.195] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2018] [Revised: 02/19/2019] [Accepted: 02/20/2019] [Indexed: 11/30/2022]
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ACR Appropriateness Criteria® Neuroendocrine Imaging. J Am Coll Radiol 2019; 16:S161-S173. [DOI: 10.1016/j.jacr.2019.02.017] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2019] [Accepted: 02/08/2019] [Indexed: 01/06/2023]
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The diaphragma sellae, diaphragm opening, and subdiaphragmatic cistern: an anatomical study using magnetic resonance imaging. Surg Radiol Anat 2019; 41:529-534. [DOI: 10.1007/s00276-019-02184-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2018] [Accepted: 01/10/2019] [Indexed: 11/27/2022]
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Orhan K, Aksoy S, Avsever IH, Gündüz K. Incidental Findings in TMJ Imaging. IMAGING OF THE TEMPOROMANDIBULAR JOINT 2019. [PMCID: PMC7115005 DOI: 10.1007/978-3-319-99468-0_11] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
For years, the conventional imaging modalities are generally used in dental practice routine. They provide precious information about dentomaxillofacial region; they also have some limitations such as superimpositions, magnifications, distortions, and low-image quality. Because of these limitations, some pathologies, calcifications, and anatomical variations may be missed. Any findings on a radiographic image which are not related to the research areas of interest could be a description of “incidental findings.” Calcifications are one of these incidental findings. They are asymptomatic and also common in TMJ images. Although most of the calcifications require no treatment, correct identification will reduce further diagnostic assessments. Incidental findings on TMJ images were rare, while examining images for temporomandibular disorders and associated issues, it is important not only to examine the TMJ structures but also to look at the nearby anatomical features to check for evidence of incidental findings pathologies that may have mimicked signs and symptoms of TMJ disorders.
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45
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Anyiam O, Cheung B, Al‐sabbagh S. Subclinical hypothyroidism or central hypothyroidism-The danger of thyroid function misinterpretation. Clin Case Rep 2018; 6:1953-1957. [PMID: 30349705 PMCID: PMC6186879 DOI: 10.1002/ccr3.1694] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2018] [Revised: 03/23/2018] [Accepted: 05/25/2018] [Indexed: 01/28/2023] Open
Abstract
Correct interpretation of thyroid function tests is critical to providing appropriate care to patients with suspected thyroid disease. It is particularly important to distinguish central hypothyroidism from other types due to the potential of concurrent secondary adrenal insufficiency and thus the need for immediate steroid replacement prior to commencing thyroxine.
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Affiliation(s)
| | - Billy Cheung
- East and North Hertfordshire NHS TrustStevenageHertfordshireUK
- Present address:
Homerton University HospitalLondonUK
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K. Auer M, R. Stieg M, Crispin A, Sievers C, K. Stalla G, Kopczak A. Primary Empty Sella Syndrome and the Prevalence of Hormonal Dysregulation. DEUTSCHES ARZTEBLATT INTERNATIONAL 2018; 115:99-105. [PMID: 29510819 PMCID: PMC5842341 DOI: 10.3238/arztebl.2018.0099] [Citation(s) in RCA: 30] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/30/2017] [Revised: 05/30/2017] [Accepted: 11/08/2017] [Indexed: 11/27/2022]
Abstract
BACKGROUND Empty sella is the neuroradiological or pathological finding of an apparently empty sella turcica containing no pituitary tissue. The prevalence of primary empty sella, i.e., empty sella without any discernible cause, is not precisely known; estimates range from 2% to 20%. Technical advances in neuroradiology have made empty sella an increasingly common incidental finding. It remains unclear whether, and to what extent, asymptomatic adult patients with an incidentally discovered empty sella should undergo diagnostic testing for hormonal disturbances. METHODS To answer this question, the authors carried out a systematic search in the PubMed and Web of Science databases for publications that appeared in the period 1995-2016 and that contained the search term "empty sella" (registration: PROSPERO 2015: CRD42015024550). RESULTS The search yielded 1282 hits. After the exclusion of duplicates, pediatric reports, case reports, and veterinary studies, 120 publications on primary empty sella syndrome (PES) were identified. 4 of these dealt with the prevalence of pituitary insufficiency in patients with PES as an incidental finding. Among patients with PES, the relative frequency of pituitary insufficiency in the pooled analysis was 52% (95% confidence interval [38; 65]). CONCLUSION The data on PES as an incidental finding are too sparse to enable any evidence-based recommendation on the potential indications for hormone testing or its nature and extent. We advise basic neuroendocrinological testing (fasting cortisol, free thyroxine [fT4], estradiol or testosterone, insulin-like growth factor 1 [IGF-1], and prolactin). There is an unexplained discrepancy between the reported high prevalence of pituitary insufficiency among persons with PES and its low prevalence in epidemiologic studies. We suspect that the former may be high because of selection bias in the publications that we reviewed, or else the latter may be erroneously low.
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Affiliation(s)
- Matthias K. Auer
- Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich
- Department of Medicine IV, Ludwig-Maximilians-Universität München, Munich
| | - Mareike R. Stieg
- Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich
- Department of Medicine IV, Ludwig-Maximilians-Universität München, Munich
| | - Alexander Crispin
- Institute for Medical Data Processing, Biometrics and Epidemiology, Ludwig-Maximilians-Universität München, Munich
| | - Caroline Sievers
- Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich
| | - Günter K. Stalla
- Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich
| | - Anna Kopczak
- Department of Clinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich
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Barzaghi LR, Donofrio CA, Panni P, Losa M, Mortini P. Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques. Pituitary 2018; 21:98-106. [PMID: 29027644 DOI: 10.1007/s11102-017-0842-6] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
PURPOSE Chiasmapexy is a poorly described surgical procedure adopted to correct the downward displacement of suprasellar visual system (SVS) into an empty sella (ES) causing visual worsening. The aim of our study is to define the indications for extradural and intradural chiasmapexy. METHODS A systematic literature review has been performed on MEDLINE database (US National Library of Medicine), including only articles that depicted cases of surgically treated patients affected by ES and progressive delayed visual worsening. Moreover, we have reported three cases of secondary ES syndrome (SESS) with visual worsening treated in our Department with transsphenoidal (TS) microsurgical intradural approach. Finally, we have compared the results of extradural and intradural chiasmapexy described in literature. RESULTS The etiology of visual impairment is different in primary and secondary ESS. In primary ESS (PESS) the only predisposing factor is a dehiscence of diaphragma sellae, and the anatomical distortion caused by displacement of optic chiasm or traction of pituitary stalk and infundibulum may determine a direct injury of neural fibers and ischemic damage of SVS. In PESS the mechanical elevation of SVS performed through extradural approach is sufficient to resolve the main pathologic mechanism. In SESS, arachnoidal adhesions play an important role in addition to downward herniation of SVS. Consequently, the surgical technique should provide elevation of SVS combined to intradural release of scar tissue and arachnoidal adhesions. In treatment of SESS, the intradural approaches result to be more effective, guaranteeing the best visual outcomes with the lowest complications rates. CONCLUSIONS The intradural chiasmapexy is indicated in treatment of SESS, instead the extradural approaches are suggested for surgical management of PESS.
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Affiliation(s)
- Lina Raffaella Barzaghi
- Pituitary Unit of the Department of Neurosurgery and Gamma Knife Radiosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina 60, 20132, Milan, Italy
| | - Carmine Antonio Donofrio
- Pituitary Unit of the Department of Neurosurgery and Gamma Knife Radiosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina 60, 20132, Milan, Italy.
| | - Pietro Panni
- Pituitary Unit of the Department of Neurosurgery and Gamma Knife Radiosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina 60, 20132, Milan, Italy
| | - Marco Losa
- Pituitary Unit of the Department of Neurosurgery and Gamma Knife Radiosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina 60, 20132, Milan, Italy
| | - Pietro Mortini
- Pituitary Unit of the Department of Neurosurgery and Gamma Knife Radiosurgery, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina 60, 20132, Milan, Italy
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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:R275-R285. [PMID: 28780516 DOI: 10.1530/eje-17-0505] [Citation(s) in RCA: 87] [Impact Index Per Article: 10.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2017] [Revised: 07/23/2017] [Accepted: 07/31/2017] [Indexed: 01/23/2023]
Abstract
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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Affiliation(s)
- S Chiloiro
- Pituitary Unit, Department of Endocrinology
| | | | - A Bianchi
- Pituitary Unit, Department of Endocrinology
| | | | | | - C Anile
- Institute of Neurosurgery, Catholic University of the Sacred Heart, Agostino Gemelli Foundation, Rome, Italy
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Lee MH, Calder GL, MacIsaac RJ, Sachithanandan N. Hyponatraemia and hypopituitarism: an easily missed entity. Med J Aust 2017; 207:282-283. [DOI: 10.5694/mja16.00921] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2016] [Accepted: 02/23/2017] [Indexed: 11/17/2022]
Affiliation(s)
| | | | - Richard J MacIsaac
- St Vincent's Hospital, Melbourne, VIC
- University of Melbourne, Melbourne, VIC
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Wang Q, Guo X, Gao L, Wang Z, Deng K, Lian W, Wang R, Zhu H, Xing B. Surgical Outcome of Growth Hormone–Secreting Pituitary Adenoma with Empty Sella Using a New Classification. World Neurosurg 2017. [DOI: 10.1016/j.wneu.2017.06.071] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
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