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Empty Sella Syndrome - Types, Symptoms, Diagnosis, and Treatment

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Empty sella syndrome is when sella turcica, a bony part in the skull, gets filled with cerebrospinal fluid. Read the article to know more about it.

Medically reviewed by

Dr. Shaikh Sadaf

Published At December 20, 2022
Reviewed AtJanuary 29, 2024

Introduction:

Sella turcica is an indentation present in the sphenoid bone, a bone in the front part of the skull. Sella turcica houses the pituitary gland, also known as the "master gland." The pituitary gland works along with the hypothalamus, forming the hypothalamus-pituitary complex and releasing various hormones into the bloodstream, which act on different body parts.

What Is Empty Sella Syndrome?

Empty sella syndrome is a rare disorder in which sella turcica, a small bony cavity in the sphenoid bone, gets filled with cerebrospinal fluid (CSF). The sella turcica contains the pituitary gland. Empty sella does not mean the cavity is empty but partially or entirely with CSF. The pituitary gland is also found to be very small or flattened. The size of the gland is generally noted in imaging tests. Sometimes, the gland is so small that it goes undetected on the scans (imaging tests). Normal MRI (magnetic resonance imaging) scan of the brain shows the pituitary gland occupying almost the entire sella turcica. However, in empty sella syndrome, the pituitary gland shrinks in size, which can decrease the hormones released by the gland.

This condition is called hypopituitarism, which affects the levels of the following hormones released by the pituitary:

  • Growth hormone (GH).

  • Adrenocorticotropic hormone (ACTH).

  • Thyroid-stimulating hormone (TSH).

  • Follicle-stimulating hormone (FSH).

  • Luteinizing hormone (LH).

  • Prolactin (PRL).

  • Oxytocin.

  • Antidiuretic hormone (ADH) or vasopressin.

What Are the Other Names of Empty Sella Syndrome?

The alternative names of the condition include:

  • Pituitary-empty sella syndrome.

  • Partial empty sella.

  • ESS.

What Are the Types of Empty Sella Syndrome?

Based on the cause, empty sella syndrome is of two types, namely:

1) Primary Empty Sella Syndrome - The exact cause of primary empty sella syndrome is not known. However, doctors believe that a defect or a tear in a membrane called diaphragma sellae causes the CSF to leak into the sella turcica. This defect is found to be present since birth. However, it is still unsure whether this is the exact cause or only a risk factor for empty sella syndrome. Also, another study says that empty sella syndrome can occur when the arachnoid mater (one of the brain's outer layers) bulges into sella turcica, flattening the pituitary gland.

Empty sella syndrome occurs four times more commonly in women when compared to men. Also, obese, middle-aged women with high blood pressure are more prone to develop the condition.

2) Secondary Empty Sella Syndrome - Secondary empty sella syndrome occurs due to damage to the pituitary gland, making the sella turcica empty. The causes of pituitary damage include the following:

  • Infection.

  • Trauma to the head.

  • Tumors of the pituitary.

  • Radiation therapy.

  • Surgery of the pituitary gland.

  • Conditions that affect the brain or pituitary, like intracranial hypertension or pseudotumor cerebri (increase in pressure inside the skull, causing severe headache and loss of vision).

  • Neurosarcoidosis (an inflammatory disorder affecting the nervous system),

  • Hypophysitis (inflammation of the pituitary gland),

  • Sheehan syndrome (damage to the pituitary gland in women during childbirth).

What Are the Symptoms of Empty Sella Syndrome?

People with empty sella syndrome do not show any symptoms in most cases. However, those having the condition due to secondary causes can have symptoms associated with the cause. Many people also experience headaches; however, doctors are unsure whether it occurs due to empty sella syndrome or high blood pressure, which is a common feature.

In rare cases, a pressure buildup in the skull can cause symptoms like:

  • Leakage of CSF from the nose.

  • Pressure on the optic nerve.

  • Vision problems.

If symptoms occur, they include:

  • Headaches.

  • Tiredness.

  • Problems in the erection.

  • Irregular or no menstrual cycle.

  • Loss of libido (decreased or complete absence of sexual desire).

  • Discharge from the nipple.

What Are the Complications of Empty Sella Syndrome?

Complications of empty sella syndrome include an elevation in the prolactin levels. Prolactin is a hormone released by the pituitary gland which promotes breast development and the production of milk in women.

Complications of secondary empty sella syndrome occur due to the cause or a deficiency in the pituitary hormones, a condition known as hypopituitarism.

How Is Empty Sella Syndrome Diagnosed?

As empty sella syndrome does not have any noticeable symptoms, diagnosis is usually made only during scans for other conditions. If the doctor suspects a person has the syndrome, they do a physical examination and take down the medical history and symptoms. Further, they may also order imaging tests like CT (computerized tomography) or MRI (magnetic resonance imaging) scans.

Imaging tests help the doctor assess if the person has partial or total empty sella syndrome.

Partial empty sella syndrome means that the sella turcica is less than half-filled with CSF, and the size of the pituitary gland is 3 to 7 mm (millimeters) thick. In complete empty sella syndrome, sella turcica is more than half-filled with CSF, with the pituitary gland being 2 mm or less in thickness. However, in most patients, the pituitary gland function is not compromised.

How Are the Symptoms of Empty Sella Syndrome Managed?

As empty sella syndrome does not present with symptoms, treatment is not required. However, patients who show signs are treated accordingly. Also, in patients with secondary empty sella syndrome, the underlying cause is treated. Surgery is indicated in cases where there is a CSF leakage from the nose, a condition called CSF rhinorrhea. Medications like Ibuprofen help relieve headaches. In cases where the pituitary gland is affected, necessary hormone replacement for deficient hormones is planned.

Conclusion:

Empty sella syndrome is a rare condition in which sella turcica, a small indentation that houses the pituitary gland in the brain, gets filled with cerebrospinal fluid. In most cases, the condition does not show any symptoms; however, in very few cases, there may be specific symptoms that occur due to the shrinkage of the pituitary gland. As the pituitary gland plays a pivotal role in various essential body functions, diagnosing the condition and starting the necessary treatment are necessary. Empty sella syndrome does not cause any adverse effects on the body; however, doctors need to identify the underlying cause and start the treatment required to improve the patient’s quality of life.

Dr. Shaikh Sadaf
Dr. Shaikh Sadaf

Endocrinology

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