Empty Sella Syndrome

What is empty sella syndrome (ESS)?

Empty sella syndrome (ESS), also called arachnoidocele, is a rare condition that affects the pituitary gland. The pituitary gland is a small organ about the size of a kidney bean. It controls important body functions by releasing hormones into the bloodstream. Growth hormone controls the growth of bones and muscles during childhood. The thyroid-stimulating hormone controls the thyroid gland and keeps the body’s energy levels steady. Other hormones regulate stress, water balance, and reproduction. Together, these hormones help the body function properly. Normally, the pituitary gland fills a spot called the sella turcica (‘Turkish saddle’ in Latin). Modern medical imaging shows the gland filling the sella completely. Empty sella syndrome (or ESS) is diagnosed when the sella appears empty on imaging. Instead of showing the pituitary gland, the sella appears to be filled with cerebrospinal fluid (or CSF). 

There are two types of ESS: 

1. Primary empty sella syndrome (ESS) is diagnosed when no underlying cause for ESS is discovered. Primary ESS is found most commonly in women who have had multiple children or people with obesity. Some patients with primary ESS can have higher levels of prolactin, a hormone that can affect how the ovaries and testes work. About half of the men with primary ESS will have low testosterone (central hypogonadism). Some patients with primary ESS may have reduced levels of pituitary hormones. Symptoms from hormone deficiencies can be treated with hormone supplements.
2. Secondary ESS occurs when the pituitary gland shrinks due to a tumor, surgery, or radiation therapy. All these events cause injury to the pituitary gland, causing it to shrink and disappear on imaging. Many people with secondary ESS have problems with pituitary hormone levels. About half of women with secondary ESS will have low growth hormone and low stress hormone levels. This loss of function can cause symptoms like:
   • Menstrual periods stopping
   • Infertility
   • Fatigue
   • Trouble handling stress and infections

While it is very rare, ESS does sometimes develop in children. In some cases, children are born with structural problems in the sella turcica. Secondary ESS in children can develop due to trauma, tumors,  cysts, or chronic illnesses. Many children with ESS have no symptoms. However, some may experience early puberty, growth delays, hormone imbalances, or vision problems. 

How is empty sella syndrome diagnosed and treated?

Diagnosing Empty Sella Syndrome (ESS)

Doctors often use MRI (magnetic resonance imaging) or CT (computed tomography) scans to diagnose ESS. ESS is most often diagnosed when the doctors order MRI or CT for other reasons. If some of the gland is visible within the sella, a ‘partial’ empty sella is diagnosed. If the person’s symptoms point to a hormone imbalance, blood tests can measure the levels of pituitary hormones.  

Treating Empty Sella Syndrome (ESS)

ESS is not a life-threatening condition. If they don’t have symptoms, people with ESS do not need treatment. Regular checkups can help doctors monitor the condition over time. However, if hormone levels are low or ESS results in other medical problems, treatments can help with their symptoms. 

Treatment may include medications, such as hormone replacement therapy to restore missing hormones or other medications to correct hormone imbalances. People with obesity may be able to reduce their symptoms through lifestyle changes to manage body weight. In rare cases, surgery to place a shunt (a small tube) may be used to relieve pressure on the brain.

How can I or my loved one help improve care for people with empty sella syndrome?

Consider participating in a clinical trial so clinicians and scientists can learn more about ESS and related disorders. Clinical research with human study participants to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of participants are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with ESS at Clinicaltrials.gov.

Learn about clinical trials currently looking for people with ESS at ClinicalTrials.gov, a searchable database of current and past clinical studies and research results.