Empty Sella Syndrome: Types, Symptoms, Causes and Treatments

Overview

Empty Sella Syndrome (ESS) is a neurological disorder in which the pituitary gland either gets flattened or shrinks due to the enlargement of the sella turcica. It is a bony structure that surrounds and protects the pituitary gland and is present at the base of the brain. The disorder gets its name because, during imaging tests of the sella turcica, the shell may appear to be empty. Empty Sella Syndrome causes symptoms such as hormonal imbalances, frequent headaches, and vision problems. 

The blog explains Empty Sella Syndrome, its symptoms, types, causes, and various treatment options.

What is empty sella syndrome?

In a human skull, a small, bony nook is at the base of the brain that surrounds, holds, and protects the pituitary gland – it controls and regulates the various hormones in a body. The tiny structure is the sella turcica, which gets its name from a Latin word,  ‘Turkish seat.’ 

In a few people, the sella turcica is developed in a manner where the spinal fluid can easily enter the structure. The build of the spinal fluid flattens the pituitary gland making it look like the sella turcica is empty. Doctors often detect this disorder when looking for causes of other problems.

ESS is more likely to develop in women than men. People with high blood pressure and suffering from obesity are also at a higher risk of developing ESS.

What are the types of empty sella syndrome?

When a doctor diagnoses a patient with Empty Sella Syndrome, they categorize the disorder into two categories. The two types of ESS are as follows:

1. Primary ESS: Typically, in this, the pituitary gland becomes flat. It is common in women who are obese and suffer from high blood pressure. Primary ESS is linked to fluid buildup in the brain. In a few people, the sella turcica is developed in a manner where the spinal fluid can easily enter the structure. The build of the spinal fluid flattens the pituitary gland making it look like the sella turcica is empty. It can be noticed in patients with pseudotumor cerebii. 
2. Secondary ESS: The disorder is due to an identifiable underlying condition which changes the pituitary gland’s anatomy. The usual reason for certain kinds of damage to the pituitary gland may be due to genetic mutation, injury, radiation therapy, or surgery. 

What are the symptoms of empty sella syndrome?

Often Empty Sella Symptoms do not show any signs or symptoms. Depending on age, symptoms may differ from person to person, and doctors believe only 1% of patients show symptoms. If a patient notices symptoms, they may be as follows:

• Impotence in men
• Reduced sexual desire
• Irregular or no menstrual periods in women
• Chronic headaches
• High blood pressure
• Fatigue
• Infertility
• Hormonal imbalance
• Erectile dysfunction
• Galactorrhea – irregular nipple discharge

Some of the lesser-known symptoms include the following:

• A feeling of pressure within the skull
• A spinal fluid that leaks from the nose
• Swelling of eyes
• Blurry vision

The function of the pituitary gland in the empty sella syndrome is generally not affected. While it’s often not well seen on imaging, it is perfectly functional otherwise. The pituitary is a small gland situated close to the base of your skull that stores numerious critical hormones and releases these harmones into the bloodstream as required by the body. These hormones regulate various different functions of the body. Although a rarely, some decreased or abnormal pituitary function can happen (hypopituitarism) in the setting of empty sella. A detailed finding in some people with empty sella, like children, has been the isolated growth hormone deficiency.

When to seek medical advice?

Consult a doctor if the symptoms worsen or have newer symptoms. 

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What are the causes of empty sella syndrome?

The causes of primary and secondary Empty Sella Syndrome are different. The cause of primary ESS is unknown. However, healthcare providers believe it is due to a congenital disability. 

The reason for secondary ESS is due to damage in the sella turcica as a result of other health conditions. The following are the causes of the damage:

• A brain tumour: Occasionally, brain tumours increase brain pressure leading to herniation of the subarachnoid space. Thus, resulting in compression of the pituitary gland.
• Idiopathic intracranial hypertension: When the cerebrospinal fluid builds up in the brain, idiopathic intracranial hypertension increase pressure in the skull. The increased pressure leads to compression of the pituitary gland. 
• Pituitary adenomas: A growth or a tumour on the pituitary gland is known as a pituitary adenoma. It is often noncancerous. The tumour puts pressure on the pituitary gland and damages it. 
• Sheehan’s syndrome: People who lose a significant amount of blood during childbirth leading to oxygen deprivation and a life-threatening event, may develop Sheehan’s syndrome. The lack of oxygen can cause damage to the pituitary gland.
• Radiation therapy
• Brain surgery, particularly in the pituitary gland
• Head injury, including a traumatic brain injury.

How is empty sella syndrome diagnosed?

Empty Sella Syndrome is challenging to diagnose. But it is most often found when searching for a cause for another medication. The healthcare provider takes details about medical history and conducts a physical examination. The doctor may also prescribe the following tests:

• CT scan: The doctor may take images of the skull from different angles. It helps find the root cause of the problem. 
• Magnetic resonance imaging (MRI) scan: The test uses powerful magnets and radio waves to get detailed brain images. 

In rare cases, the patient may show symptoms of ESS. In such cases, the doctor may order several tests, including the following:

• Blood tests to check for hormonal imbalance
• Eye check to examine the retina
• Spinal tap to check the CSF. It determines if the patient has partial or total Empty Sella Syndrome.

How is empty sella syndrome treated?

Based on the age, symptoms, medical history of previous treatments or surgeries, and duration of the condition, the doctor, treats ESS. However, most cases of ESS do not require any treatment. In rare instances in which the symptoms hamper the daily life of a patient, the doctor may use the following treatment options: 

• Medicine: If the pituitary gland fails to produce the right amount of hormones efficiently, the doctor prescribes medications to fix the same. 
• Surgery: When the CSF leaks from the patient’s nose, surgery is required to stop it from happening. 

However, if a patient suffers from secondary ESS due to another medical condition, the doctor starts treating the underlying medical condition to eliminate or manage the symptoms of ESS.

Conclusion

The prognosis is good for patients with no symptoms and does not affect their overall health. If a patient has symptoms, it is easily treatable with medications and surgery. A person can lead a healthy, long life with a proper diagnosis. 

Frequently Asked Questions (FAQs)

Is ESS life-threatening?

ESS is not life-threatening. It is treatable with medications and surgery. 

Who is more likely to develop ESS?

Women are four times more likely to develop Empty Sella Syndrome than men. Primary Empty Sella Syndrom occurs between 30 to 40 years.