Empty Sella Syndrome (ESS): Symptoms, Causes, Treatment

What are the symptoms of empty sella syndrome?

Empty sella syndrome is a rare condition in which the pituitary gland is partially or completely surrounded by bone, causing compression and potential disruption of pituitary function. The symptoms of empty sella syndrome can vary depending on the severity of the compression and the extent of pituitary dysfunction. Common symptoms include:

1. Hypopituitarism: Insufficient production of one or more pituitary hormones, such as:
   • Growth hormone deficiency (short stature, delayed growth and development)
   • Adrenocorticotropic hormone (ACTH) deficiency (adrenal insufficiency, fatigue, weight loss)
   • Thyroid-stimulating hormone (TSH) deficiency (hypothyroidism)
   • Prolactin deficiency (infertility, breast milk insufficiency)
2. Visual disturbances:
   • Vision loss or blindness
   • Double vision
   • Diplopia (double vision)
   • Blind spots or blind areas
3. Headaches: Frequent or severe headaches, often accompanied by nausea and vomiting
4. Hormonal imbalances: Changes in menstrual cycles, libido, or fertility
5. Fatigue: Feeling tired or weak all the time
6. Weight gain or loss: Unexplained changes in weight due to hormonal imbalances
7. Mood changes: Mood swings, anxiety, depression, or irritability
8. Numbness or tingling: Numbness or tingling sensations in the face, arms, or legs
9. Weakness: Muscle weakness, particularly in the arms and legs
10. Difficulty walking: Gait disturbances, clumsiness, or difficulty maintaining balance

In some cases, people with empty sella syndrome may not experience any symptoms at all until the condition progresses and pituitary function is severely impaired.

It’s essential to note that empty sella syndrome can occur without symptoms until it leads to significant pituitary dysfunction. Regular monitoring by a healthcare provider and timely treatment can help manage the condition and prevent complications.

What are the causes of empty sella syndrome?

Empty sella syndrome (ESS) is a condition characterized by the enlargement or partial filling of the sella turcica, a saddle-shaped structure in the skull where the pituitary gland is located. There are two types of ESS: primary (or idiopathic) and secondary. Here are the causes of each:

1. Primary Empty Sella Syndrome: The exact cause of primary ESS is not fully understood. It is believed to be due to a defect in the sellar diaphragm, a membrane that covers the pituitary gland. This defect can lead to an increase in cerebrospinal fluid pressure, which may result in the flattening or compression of the pituitary gland against the sella floor, causing it to appear “empty.”
2. Secondary Empty Sella Syndrome: This type of ESS is caused by an underlying condition or factor that leads to the enlargement of the sella turcica. Some common causes include:

• Pituitary surgery: Previous surgery on the pituitary gland can cause the sella turcica to become empty.
• Pituitary radiation therapy: Radiation therapy to the brain, typically used to treat tumors, can damage the pituitary gland and lead to ESS.
• Pituitary apoplexy: This is a rare but serious condition where the blood supply to the pituitary gland is suddenly cut off, often due to a hemorrhage or infarction. This can cause the gland to shrink and the sella to appear empty.
• Pituitary tumors: Large tumors in the pituitary gland can compress the gland and lead to an empty sella.
• Other conditions: Some other conditions, such as meningitis, trauma to the head, or certain genetic disorders, can also contribute to the development of ESS.

It’s important to note that while ESS itself may not cause symptoms in some cases, it can sometimes be associated with hormonal imbalances or pituitary dysfunction, which may require treatment. Treatment options depend on the underlying cause and symptoms, and may include hormone replacement therapy or other interventions to manage pituitary function.

How is empty sella syndrome diagnosed?

Empty sella syndrome (ESS) is typically diagnosed through a combination of medical history, physical examination, and imaging studies. Here’s how it’s typically diagnosed:

1. Medical History and Physical Examination: Your healthcare provider will take a detailed medical history to understand your symptoms and any underlying conditions you may have. They will also perform a physical examination to check for signs of hormonal imbalances or other related issues.
2. Hormone Testing: Blood tests may be done to check for hormonal imbalances that could be associated with ESS. This may include testing for levels of hormones such as cortisol, thyroid hormones, prolactin, and others that are produced by the pituitary gland.
3. Imaging Studies: Imaging studies are crucial for diagnosing ESS. The most common imaging technique used is magnetic resonance imaging (MRI) of the brain. MRI can provide detailed images of the pituitary gland and the sella turcica, allowing healthcare providers to see if the sella is enlarged or if there are any abnormalities in the pituitary gland.
4. Visual Field Testing: If there is suspicion of optic nerve compression due to ESS, visual field testing may be performed to assess any potential visual field defects.
5. Other Tests: Depending on the symptoms and suspected underlying causes, additional tests such as a lumbar puncture (to measure cerebrospinal fluid pressure) or genetic testing may be done.
6. Differential Diagnosis: ESS must be differentiated from other conditions that can cause similar symptoms or imaging findings, such as pituitary tumors, Rathke’s cleft cysts, or other sellar or suprasellar masses.

If ESS is diagnosed, further evaluation may be needed to determine if there are any associated hormonal imbalances or pituitary dysfunction that require treatment. Treatment options depend on the underlying cause and symptoms and may include hormone replacement therapy or other interventions to manage pituitary function.

What is the treatment for empty sella syndrome?

The treatment for empty sella syndrome (ESS) depends on the underlying cause and whether the condition is causing symptoms. Here are the general approaches to managing ESS:

1. Monitoring: If ESS is not causing symptoms and there are no hormonal imbalances, your healthcare provider may recommend regular monitoring with periodic imaging studies and hormone tests to check for any changes.
2. Hormone Replacement Therapy: If ESS is associated with hormonal imbalances due to pituitary dysfunction, hormone replacement therapy may be prescribed. This could include replacement of hormones such as cortisol, thyroid hormones, or sex hormones.
3. Management of Symptoms: If ESS is causing symptoms such as headaches or visual disturbances, medications may be prescribed to manage these symptoms. For example, pain relievers for headaches or medications to reduce cerebrospinal fluid pressure.
4. Treatment of Underlying Conditions: If ESS is secondary to an underlying condition such as a pituitary tumor, treatment will focus on managing that condition. This may involve surgery, radiation therapy, or medications to shrink or control the tumor.
5. Regular Follow-up: Regular follow-up with your healthcare provider is important to monitor the condition and adjust treatment as needed.
6. Lifestyle Modifications: In some cases, lifestyle modifications such as maintaining a healthy weight, managing stress, and getting regular exercise may be beneficial in managing ESS.

It’s important to work closely with your healthcare provider to determine the most appropriate treatment plan for your specific situation. Treatment decisions will depend on the underlying cause of ESS, the presence of symptoms, and other individual factors.