Empty sella syndrome

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Empty sella syndrome
Other namesPituitary - empty sella syndrome [1]
Empty sella MRI.png
MRI of Empty Sella
Specialty Endocrinology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Cryptorchidism
CausesArachnoid presses down on gland (another possibility is a Tumor, Radiation therapy) [1]
Diagnostic method MRI, CT scan [1]
Medication Manage abnormal hormone levels [1]

Empty sella syndrome is the condition when the pituitary gland shrinks or becomes flattened, filling the sella turcica with cerebrospinal fluid instead of the normal pituitary. [2] It can be discovered as part of the diagnostic workup of pituitary disorders, or as an incidental finding when imaging the brain. [1]

Contents

Signs and symptoms

If there are symptoms, people with empty sella syndrome can have headaches and vision loss. Additional symptoms would be associated with hypopituitarism. [3] [4] Additional symptoms are as follows:[ citation needed ]

Cause

Pituitary gland Pituitary gland small.gif
Pituitary gland

The cause of this condition is divided into primary and secondary, as follows:

Mechanism

The normal mechanism of the pituitary gland sees that it controls the hormonal system, which therefore has an effect on growth, sexual development, and adrenocortical function. The gland is divided into anterior and posterior. [7]

Its pathophysiology is such that individuals affected with the condition can have cerebrospinal fluid build-up, which in turn causes intracranial pressure leading to headaches for the individual. [8]

Diagnosis

Empty sella in MRI Empty Sella MRT T2 sag 002.jpg
Empty sella in MRI

The diagnosis of empty sella syndrome, done via examination (and test), may be linked to early onset of puberty, growth hormone deficiency, or pituitary gland dysfunction (at an early age). [2] Additionally there is:

Classification

There are two types of empty sella syndrome: primary and secondary.

Differential diagnosis

The major differential to consider in empty sella syndrome is intracranial hypertension, of both unknown and secondary causes, and an epidermoid cyst, which can mimic cerebrospinal fluid due to its low density on CT scans, although MRI can usually distinguish the latter diagnosis. [10]

Treatment

In terms of management, unless the syndrome results in other medical problems, treatment for endocrine dysfunction associated with pituitary malfunction is symptomatic and thus supportive; however, surgery may be needed in some cases. [2]

Related Research Articles

<span class="mw-page-title-main">Idiopathic intracranial hypertension</span> Medical condition

Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure without a detectable cause. The main symptoms are headache, vision problems, ringing in the ears, and shoulder pain. Complications may include vision loss.

<span class="mw-page-title-main">Syringomyelia</span> Disorder in which a cyst forms in the spinal cord

Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord. The damage may result in loss of feeling, paralysis, weakness, and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. It may also lead to a cape-like bilateral loss of pain and temperature sensation along the upper chest and arms. The combination of symptoms varies from one patient to another depending on the location of the syrinx within the spinal cord, as well as its extent.

<span class="mw-page-title-main">Cushing's syndrome</span> Symptoms from excessive exposure to glucocorticoids such as cortisol

Cushing's syndrome is a collection of signs and symptoms due to prolonged exposure to glucocorticoids such as cortisol. Signs and symptoms may include high blood pressure, abdominal obesity but with thin arms and legs, reddish stretch marks, a round red face due to facial plethora, a fat lump between the shoulders, weak muscles, weak bones, acne, and fragile skin that heals poorly. Women may have more hair and irregular menstruation. Occasionally there may be changes in mood, headaches, and a chronic feeling of tiredness.

<span class="mw-page-title-main">Adrenal insufficiency</span> Medical condition

Adrenal insufficiency is a condition in which the adrenal glands do not produce adequate amounts of steroid hormones. The adrenal glands—also referred to as the adrenal cortex—normally secrete glucocorticoids, mineralocorticoids, and androgens. These hormones are important in regulating blood pressure, electrolytes, and metabolism as a whole. Deficiency of these hormones leads to symptoms ranging from abdominal pain, vomiting, muscle weakness and fatigue, low blood pressure, depression, mood and personality changes to organ failure and shock. Adrenal crisis may occur if a person having adrenal insufficiency experiences stresses, such as an accident, injury, surgery, or severe infection; this is a life-threatening medical condition resulting from severe deficiency of cortisol in the body. Death may quickly follow.

<span class="mw-page-title-main">Sella turcica</span> Saddle-shaped depression in the sphenoid bone of the skull

The sella turcica is a saddle-shaped depression in the body of the sphenoid bone of the human skull and of the skulls of other hominids including chimpanzees, gorillas and orangutans. It serves as a cephalometric landmark. The pituitary gland or hypophysis is located within the most inferior aspect of the sella turcica, the hypophyseal fossa.

<span class="mw-page-title-main">Sheehan's syndrome</span> Medical condition

Sheehan's syndrome, also known as postpartum pituitary gland necrosis, occurs when the pituitary gland is damaged due to significant blood loss and hypovolemic shock usually during or after childbirth leading to decreased functioning of the pituitary gland (hypopituitarism). The pituitary gland is an endocrine organ, meaning it produces certain hormones and is involved in the regulation of various other hormones. This gland is located in the brain and sits in a pocket of the sphenoid bone known as the sella turcica. The pituitary gland works in conjunction with the hypothalamus, and other endocrine organs to modulate numerous bodily functions including growth, metabolism, menstruation, lactation, and even the "fight-or-flight" response. These endocrine organs release hormones in very specific pathways, known as hormonal axes. For example, the release of a hormone in the hypothalamus will target the pituitary to trigger the release of a subsequent hormone, and the pituitary's released hormone will target the next organ in the pathway. Hence, damage to the pituitary gland can have downstream effects on any of the aforementioned bodily functions.

<span class="mw-page-title-main">Pituitary adenoma</span> Human disease

Pituitary adenomas are tumors that occur in the pituitary gland. Most pituitary tumors are benign, approximately 35% are invasive and just 0.1% to 0.2% are carcinomas. Pituitary adenomas represent from 10% to 25% of all intracranial neoplasms and the estimated prevalence rate in the general population is approximately 17%.

A thunderclap headache is a headache that is severe and has a sudden onset. It is defined as a severe headache that takes seconds to minutes to reach maximum intensity. Although approximately 75% are attributed to "primary" headaches—headache disorder, non-specific headache, idiopathic thunderclap headache, or uncertain headache disorder—the remainder are secondary to other causes, which can include some extremely dangerous acute conditions, as well as infections and other conditions. Usually, further investigations are performed to identify the underlying cause.

<span class="mw-page-title-main">Hypoaldosteronism</span> Medical condition

Hypoaldosteronism is an endocrinological disorder characterized by decreased levels of the hormone aldosterone. Similarly, isolated hypoaldosteronism is the condition of having lowered aldosterone without corresponding changes in cortisol.

<span class="mw-page-title-main">Arachnoid cyst</span> Medical condition

Arachnoid cysts are cerebrospinal fluid covered by arachnoidal cells and collagen that may develop between the surface of the brain and the cranial base or on the arachnoid membrane, one of the three meningeal layers that cover the brain and the spinal cord. Primary arachnoid cysts are a congenital disorder whereas secondary arachnoid cysts are the result of head injury or trauma. Most cases of primary cysts begin during infancy; however, onset may be delayed until adolescence.

Nelson's syndrome is a disorder that occurs in about one in four patients who have had both adrenal glands removed to treat Cushing's disease. In patients with pre-existing adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas, loss of adrenal feedback following bilateral adrenalectomy can trigger the rapid growth of the tumor, leading to visual symptoms and hyperpigmentation. The severity of the disease is dependent upon the effect of ACTH release on the skin, pituitary hormone loss from mass compression, as well as invasion into surrounding structures around the pituitary gland.

<span class="mw-page-title-main">Hyperpituitarism</span> Medical condition

Hyperpituitarism is a condition due to the primary hypersecretion of pituitary hormones; it typically results from a pituitary adenoma. In children with hyperpituitarism, disruption of growth regulation is rare, either because of hormone hypersecretion or because of manifestations caused by local compression of the adenoma.

Pituitary apoplexy is bleeding into or impaired blood supply of the pituitary gland. This usually occurs in the presence of a tumor of the pituitary, although in 80% of cases this has not been diagnosed previously. The most common initial symptom is a sudden headache, often associated with a rapidly worsening visual field defect or double vision caused by compression of nerves surrounding the gland. This is often followed by acute symptoms caused by lack of secretion of essential hormones, predominantly adrenal insufficiency.

In medicine, a mass effect is the effect of a growing mass that results in secondary pathological effects by pushing on or displacing surrounding tissue.

The cerebellopontine angle syndrome is a distinct neurological syndrome of deficits that can arise due to the closeness of the cerebellopontine angle to specific cranial nerves. Indications include unilateral hearing loss (85%), speech impediments, disequilibrium, tremors or other loss of motor control. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine angle that lies between the cerebellum and the pons. It is filled with cerebrospinal fluid and is a common site for the growth of acoustic neuromas or schwannomas.

<span class="mw-page-title-main">Pineocytoma</span> Medical condition

Pineocytoma, is a rare, benign, slowly growing tumor of the pineal gland. The pineal gland is a small endocrine gland close to the center of the brain that secretes melatonin into the bloodstream. Pineocytomas can cause pressure and fluid build-up in the brain. They are more common in adults. Symptoms include vision problems, nausea, vomiting, memory problems, and headaches.

<span class="mw-page-title-main">Orthostatic headache</span> Medical condition

Orthostatic headache is a medical condition in which a person develops a headache while vertical and the headache is relieved when horizontal. Previously it was often misdiagnosed as different primary headache disorders such as migraine or tension headaches. Increasing awareness of the symptom and its causes has prevented delayed or missed diagnosis.

The International Classification of Headache Disorders (ICHD) is a detailed hierarchical classification of all headache-related disorders published by the International Headache Society. It is considered the official classification of headaches by the World Health Organization, and, in 1992, was incorporated into the 10th edition of their International Classification of Diseases (ICD-10). Each class of headache contains explicit diagnostic criteria—meaning that the criteria include quantities rather than vague terms like several or usually—that are based on clinical and laboratory observations.

<span class="mw-page-title-main">Cerebrospinal fluid leak</span> Medical condition

A cerebrospinal fluid leak is a medical condition where the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord leaks out of one or more holes or tears in the dura mater. A CSF leak is classed as either nonspontaneous (primary), having a known cause, or spontaneous (secondary) where the cause is not readily evident. Causes of a primary CSF leak are those of trauma including from an accident or intentional injury, or arising from a medical intervention known as iatrogenic. A basilar skull fracture as a cause can give the sign of CSF leakage from the ear nose or mouth. A lumbar puncture can give the symptom of a post-dural-puncture headache.

<span class="mw-page-title-main">Hypophysitis</span> Medical condition

Hypophysitis refers to an inflammation of the pituitary gland. Hypophysitis is rare and not fully understood.

References

  1. 1 2 3 4 5 6 MedlinePlus Encyclopedia : Empty sella syndrome
  2. 1 2 3 "Empty Sella Syndrome Information Page". www.ninds.nih.gov. National Institute of Neurological Disorders and Stroke. Retrieved 2017-03-05.
  3. 1 2 3 4 "Empty sella syndrome". Genetic and Rare Diseases Information Center.
  4. Goldman L, Schafer AI (2012). Goldman's Cecil Medicine (24th ed.). Elsevier Health Sciences. p. 1256. ISBN   978-1-4377-1604-7 . Retrieved 8 March 2017.
  5. Ranganathan S, Lee SH, Checkver A, Sklar E, Lam BL, Danton GH, Alperin N (August 2013). "Magnetic resonance imaging finding of empty sella in obesity related idiopathic intracranial hypertension is associated with enlarged sella turcica". Neuroradiology. 55 (8): 955–961. doi:10.1007/s00234-013-1207-0. PMC   3753687 . PMID   23708942.
  6. 1 2 3 National Organization for Rare Disorders (2003). NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. p. 530. ISBN   978-0-7817-3063-1 . Retrieved 11 March 2017.
  7. How does the pituitary gland work?. Institute for Quality and Efficiency in Health Care. 19 April 2018.{{cite book}}: |work= ignored (help)
  8. Noggle CA, Dean RS, Horton AM (2012-01-01). The Encyclopedia of Neuropsychological Disorders. Springer Publishing Company. p. 282. ISBN   978-0-8261-9854-9.
  9. Fouad W (1 June 2011). "Review of empty sella syndrome and its surgical management". Alexandria Journal of Medicine. 47 (2): 139–147. doi: 10.1016/j.ajme.2011.06.005 .
  10. González-Tortosa J (April 2009). "[Primary empty sella: symptoms, physiopathology, diagnosis and treatment]" [Primary empty sella: symptoms, physiopathology, diagnosis and treatment]. Neurocirugia (in Spanish). 20 (2): 132–151. doi:10.1016/s1130-1473(09)70180-0. PMID   19448958.

Further reading