Learn More About the Rare Condition Empty Sella Syndrome

Some of you may be unfamiliar with the term Empty Sella Syndrome. What exactly is this condition and how is it treated? Empty Sella Syndrome (ESS) is a rare medical condition involving the sella turcica , which is the bone cavity located at the base of the skull where the pituitary gland is found. The pituitary gland has a crucial role in regulating various hormonal functions of the body. In individuals with ESS, this cavity appears empty or partially filled with cerebrospinal fluid, caused by wasting or pressure on the pituitary gland.

Causes and Types of Empty Sella Syndrome

ESS can be classified into two main types, namely:

1. Primary Empty Sella Syndrome

Primary ESS occurs without a clear or detectable cause. This condition is often associated with congenital defects in the sella diaphragm, the layer of tissue that covers the sella turcica. An abnormal sella diaphragm can allow cerebrospinal fluid to enter the sella turcica, causing pressure on the pituitary gland so that it appears smaller or pushed to the side. Primary ESS is more common in women, especially those who are obese or hypertensive (high blood pressure).

2. Secondary Empty Sella Syndrome

Secondary Empty Sella Syndrome occurs due to damage to the pituitary gland which previously functioned well. Potential causes include pituitary tumors, radiation therapy, pituitary surgery, head trauma, or infections affecting the brain or pituitary gland. Compared to primary ESS, secondary ESS is sometimes more serious accompanied by more significant symptoms.

What are the symptoms of Empty Sella Syndrome?

Generally, the symptoms of ESS vary between individuals. Some people may not experience any symptoms, while others may face serious hormonal problems. The following are the symptoms caused:

  • Chronic headaches : One of the most common symptoms is headaches that are chronic and seriously interfere with daily activities.
  • Visual disturbances : This symptom occurs because the sella turcica is close to the optic nerve, pressure on the pituitary gland can affect vision.
  • Feeling tired and weak easily : Hormonal imbalances experienced by the body can cause chronic feelings of tiredness and weakness.
  • Experiencing menstrual disorders : In women, Empty Sella Syndrome can cause irregular menstruation.
  • Infertility disorders : Fertility problems can occur due to hormonal disorders that affect ovulation in women or spermatogenesis in men.
  • Thyroid problems : ESS causes hypothyroidism when the pituitary gland does not stimulate the thyroid gland enough.

How to Diagnose Empty Sella Syndrome

Diagnosis of Empty Sella Syndrome is carried out using the methods below, namely:

1. Magnetic Resonance Imaging (MRI)

The most common and effective imaging technique for detecting ESS is MRI. By utilizing magnetic fields and radio waves, MRI provides detailed images of the structure of the brain and pituitary gland.

2. Blood Test

This test can measure hormone levels in the blood and also assess the function of the pituitary gland. Blood tests can help determine if there is a hormonal imbalance that needs to be addressed.

Steps to Handle Empty Sella Syndrome

Empty Sella Syndrome treatment is given based on the symptoms and causes. Some treatment approaches include:

1. Symptom Treatment

If the patient does not experience significant symptoms or hormonal disturbances, treatment may not be necessary. Regular monitoring with imaging and blood tests may be enough to ensure the condition does not worsen.

2. Doing Hormonal Therapy

If Empty Sella Syndrome causes a hormonal imbalance, hormone replacement therapy may be needed. These can include thyroid hormone, growth hormone, corticosteroids, or sex hormones depending on which hormone is affected.

3. Treating the Underlying Cause

For secondary Empty Sella Syndrome caused by a pituitary tumor or other conditions, more intensive treatment may be needed which includes:

  • Surgical removal of the pituitary tumor if this is the cause of ESS.
  • Radiation therapy is performed to shrink pituitary tumors or prevent their growth again.
  • Treatment of infection or inflammation if Empty Sella Syndrome is caused by an infection or inflammatory condition.

4. Handling other symptoms that arise

  • Headaches can be managed with painkillers or other therapies. Of course, in this case, the use of headache medication must be accompanied by a doctor's recommendation.
  • Visual disturbances that occur require evaluation and treatment by an eye specialist.

It is now known that Empty Sella Syndrome is a rare condition that can affect the pituitary gland and the body's hormonal function. Although some patients may experience no symptoms, others may face significant health challenges. A deeper understanding of ESS, its associated symptoms, and available methods of diagnosis and treatment is essential to ensure appropriate management and improved quality of life for those affected by this condition. If you or someone you know experiences a condition that displays symptoms of Empty Sella Syndrome, consult a medical professional for immediate evaluation and further treatment.

Article written by dr. Astrid Ayodya Pattinama, Sp.N (Neurologist / Neurology Specialist at EMC Pekayon Hospital).