Pituitary imaging findings in male patients with hypogonadotrophic hypogonadism.

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Pituitary imaging findings in male patients with hypogonadotrophic hypogonadism.

Authors

Hirsch D, et al. Pituitary. 2014 Sep 23. [Epub ahead of print]

Abstract

CONTEXT: Data on pituitary imaging in adult male patients presenting with hypogonadotrophic hypogonadism (HH) and no known pituitary disease are scarce.

OBJECTIVE: To assess the usefulness of pituitary imaging in the evaluation of men presenting with HH after excluding known pituitary disorders and hyperprolactinemia.

DESIGN: A historical prospective cohort of males with HH.

PATIENTS: Men who presented for endocrine evaluation from 2011 to 2014 with testosterone levels <10.4 nmol/L (300 ng/mL), normal LH and FSH levels and no known pituitary disease.

RESULTS: Seventy-five men were included in the analysis. Their mean age and BMI were 53.4 ± 14.8 years and 30.7 ± 5.2 kg/m(2), respectively. Mean total testosterone, LH, and FSH were 6.2 ± 1.7 nmol/L, 3.4 ± 2 and 4.7 ± 3.1 mIU/L, respectively. Prolactin level within the normal range was obtained in all men (mean 161 ± 61, range 41-347 mIU/L). Sixty-two men had pituitary MRI and 13 performed CT. In 61 (81.3 %) men pituitary imaging was normal. Microadenoma was found in 8 (10.7 %), empty sella and thickened pituitary stalk in one patient (1.3 %) each. In other four patients (5.3 %) a small or mildly asymmetric pituitary gland was noted. No correlation was found between testosterone level and the presence of pituitary anomalies.

CONCLUSIONS: This study suggests that the use of routine hypothalamic-pituitary imaging in the evaluation of IHH, in the absence of clinical characteristics of other hormonal loss or sellar compression symptoms, will not increase the diagnostic yield of sellar structural abnormalities over that reported in the general population.
 
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What is Empty Sella Syndrome?​

Empty sella syndrome (ESS) is a condition where the sella turcica (the bony cavity at the base of the brain that houses the pituitary gland) appears empty or partially empty on imaging scans like MRI or CT. 145678910111213 This is because the pituitary gland is flattened or shrunken, often due to impingement by cerebrospinal fluid (CSF) that has herniated into the sella turcica. 1345678910111213There are two types of ESS:
  1. Primary ESS: This occurs when there is a congenital defect in the diaphragma sellae (the dural membrane covering the pituitary fossa), allowing CSF to herniate into the sella and compress the pituitary gland. 1345678910111213 The exact cause is unknown, but it is more common in obese middle-aged women. 124611
  2. Secondary ESS: This results from pituitary gland injury, surgery, radiation therapy, head trauma, or conditions like Sheehan's syndrome that damage the pituitary gland, causing it to shrink or become compressed by herniated CSF. 1345678910111213

Symptoms​

Many cases of ESS are asymptomatic and discovered incidentally on imaging. 1245678910111213 When symptoms occur, they may include:
  • Headaches 1246911
  • Hormone deficiencies (hypopituitarism) like growth hormone deficiency, menstrual irregularities, infertility, fatigue 124691112
  • High prolactin levels 169
  • Visual disturbances (rare) 2611
  • Cerebrospinal fluid leak from the nose (rare) 212

Diagnosis​

ESS is diagnosed by finding an empty or partially empty sella turcica on MRI or CT scans of the brain. 1245678910111213 Blood tests are done to check pituitary hormone levels. 12456789101112

Treatment​

If the pituitary gland is functioning normally, no treatment is needed for primary ESS. 12456789101112 For secondary ESS or cases with hormone deficiencies, hormone replacement therapy may be required. 2456789101112 Rarely, surgery may be needed to repair CSF leaks or decompress the pituitary gland. 212In summary, empty sella syndrome is usually an incidental radiological finding that is often asymptomatic, but can sometimes cause headaches, visual issues, or hormonal deficiencies depending on the degree of pituitary gland compression. 12345678910111213 Treatment is guided by symptoms and hormone status.

empty sella degrees.png
 
### Primary Empty Sella Syndrome (PES)

Primary empty sella syndrome (PES) is a condition characterized by the herniation of the subarachnoid space into the sella turcica, a bony structure at the base of the brain that houses the pituitary gland. This herniation often results in the flattening or shrinking of the pituitary gland, making the sella turcica appear empty on imaging studies.

#### Causes
The exact cause of PES is unknown, but it is believed to be related to a congenital defect in the diaphragma sellae, a membrane that covers the sella turcica. This defect allows cerebrospinal fluid (CSF) to leak into the sella turcica, exerting pressure on the pituitary gland and causing it to flatten or shrink[1][2][6][7].

#### Epidemiology
PES is more common in middle-aged women, particularly those who are obese and have high blood pressure. It is often discovered incidentally during imaging tests conducted for other reasons, as many individuals with PES do not exhibit symptoms[1][3][4][6].

#### Symptoms
While many individuals with PES are asymptomatic, some may experience symptoms due to hormonal imbalances or increased intracranial pressure. Common symptoms include:
- Headaches
- Visual disturbances
- Hormonal imbalances such as hyperprolactinemia and growth hormone deficiency
- Irregular menstrual periods in women
- Impotence and reduced libido in men[2][3][4][7][9].

#### Diagnosis
PES is typically diagnosed through imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans, which reveal the characteristic empty appearance of the sella turcica. Blood tests may also be conducted to assess pituitary function and check for hormonal deficiencies[3][4][6][10].

#### Treatment
Treatment for PES is generally not required if the individual is asymptomatic and has normal pituitary function. For those with hormonal deficiencies, hormone replacement therapy may be necessary. In cases where symptoms are present, a multidisciplinary approach involving endocrinologists, neurologists, and ophthalmologists is recommended to manage the condition effectively[2][3][4][6][10].

#### Prognosis
PES is not a life-threatening condition, and many individuals live without significant issues. However, ongoing monitoring and management of any hormonal imbalances or other symptoms are essential to ensure a good quality of life[3][4][6][12].

In summary, primary empty sella syndrome is a rare condition often discovered incidentally. While it can lead to various symptoms due to hormonal imbalances or increased intracranial pressure, many individuals remain asymptomatic and do not require treatment. For those with symptoms, a comprehensive and multidisciplinary approach is essential for effective management.

Citations:
[1] Empty Sella Syndrome - Symptoms, Causes, Treatment | NORD
[2] DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review - PubMed
[3] Empty Sella Syndrome (ESS): Causes, Symptoms & Treatment
[4] Empty Sella Syndrome
[5] Empty sella | Radiology Reference Article | Radiopaedia.org
[6] Empty Sella Syndrome - Pituitary Foundation
[7] Empty Sella Syndrome
[8] Empty Sella Syndrome - StatPearls - NCBI Bookshelf
[9] What Is Empty Sella Syndrome?
[10] Empty sella syndrome | You and Your Hormones from the Society for Endocrinology
[11] Empty Sella Syndrome
[12] Empty Sella Syndrome - Hormonal and Metabolic Disorders - Merck Manual Consumer Version
[13] The Radiology Assistant : Sella Turcica and Parasellar Region
[14] The Radiology Assistant : Cerebral Venous Sinus Thrombosis
[15] The Radiology Assistant : Enhancement Patterns in CNS disease
[16] The Radiology Assistant : Vascular territories of the Brain
[17] The Radiology Assistant : Müllerian duct anomalies
[18] The Radiology Assistant : Cervical injury
[19] Empty Sella Syndrome | Children's Hospital of Philadelphia
[20] The Radiology Assistant : Swallowing disorders update
 
Primary empty sella syndrome (PES) is often an incidental finding and many individuals with PES have normal pituitary function and remain asymptomatic. However, the progression of PES and its potential to worsen over time can vary based on individual circumstances.

Potential for Worsening Over Time​

  1. Hormonal Function:
    • While many individuals with PES maintain normal pituitary function, there is a possibility that hormonal deficiencies can develop over time. Studies have shown that some patients with PES may eventually experience hypopituitarism, which includes deficiencies in hormones such as growth hormone, prolactin, and others1361217.
    • A study indicated that the prevalence of endocrine abnormalities in PES necessitates prompt evaluation and monitoring, as hormonal imbalances can emerge even if they were not present initially17.
  2. Symptoms:
    • Symptoms such as headaches, visual disturbances, and other neurological issues can develop or worsen over time due to increased intracranial pressure or other factors1246812.
    • Visual disturbances, in particular, can be a concern if there is herniation of the optic chiasm into the sella turcica, which can occur in some cases of PES6.
  3. Structural Changes:
    • The anatomical changes associated with PES, such as the herniation of the subarachnoid space into the sella turcica, can potentially progress, leading to further compression of the pituitary gland and associated structures16713.

Monitoring and Management​

Given the potential for PES to worsen over time, regular monitoring is recommended. This includes:
  • Periodic Imaging: MRI or CT scans to monitor structural changes in the sella turcica and pituitary gland.
  • Endocrine Evaluation: Regular blood tests to assess pituitary function and detect any emerging hormonal deficiencies.
  • Symptom Management: Addressing any new or worsening symptoms, particularly headaches and visual disturbances, through appropriate medical interventions.

Conclusion​

While primary empty sella syndrome with normal pituitary function may remain stable in many individuals, there is a possibility for the condition to worsen over time, particularly in terms of hormonal function and symptom development. Regular monitoring and a multidisciplinary approach to management are essential to ensure timely intervention if changes occur.References:
 
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