What is empty sella syndrome?

Empty sella syndrome is a condition in which the pituitary gland is not visible or appears shrunken within the bony structure known as the sella turcica, when the brain is examined with magnetic resonance imaging (MRI) or computerized tomography (CT) scans.

The pituitary gland is a small, pea-sized gland located in the center of the brain, between and behind the eyes. It produces hormones and controls the release of hormones from other glands.

In MRI or CT scans of the brain, the pituitary gland typically fills and is visible within the sella turcica. In empty sella syndrome, the gland can appear to be shrunken or missing.

Causes of empty sella syndrome

There are several different reasons that the pituitary gland can appear to be shrunken or missing in an MRI or CT scan.

Primary empty sella syndrome

It can be because the sella turcica is unusually large and “hides” the pituitary gland. Or it can be due to pressure from the cerebrospinal fluid in the brain, which may compress or flatten the pituitary gland. These cases are classified as primary empty sella syndrome. There is no known cause for primary empty sella syndrome.

Secondary empty sella syndrome

The condition can also be due to an underdeveloped or shrunken pituitary gland as a result of injury, treatment for pituitary, tumors or the natural shrinkage of a pituitary tumor (not as a result of treatment). These cases, where the underlying cause can be identified, are classified as secondary empty sella syndrome.

Signs and symptoms of empty sella syndrome

Most people with empty sella syndrome have no symptoms. The condition is usually detected when brain imaging is done for other reasons.

A small percentage of people with empty sella syndrome experience symptoms due to reduced function in the pituitary gland or to fluid pressure in the brain (one of the causes of secondary empty sella syndrome). In these cases, symptoms may include:

• Headaches
• Vision problems
• Leakage of cerebrospinal fluid from the nose
• Abnormal slow-down of growth
• Early or delayed puberty
• Less frequent menstrual periods
• Nipple discharge
• Fatigue or low energy

Testing and diagnosis for empty sella syndrome

Empty sella syndrome is diagnosed with magnetic resonance imaging (MRI) or computerized tomography (CT) scans.

When empty sella syndrome is found with brain imaging, additional tests may be done to find out if the condition is causing any health problems. These tests may include:

• Blood tests to check for low levels of hormones
• Other examinations and tests to test for high levels of pressure in the cerebrospinal fluid surrounding the brain

Treatment for empty sella syndrome

At Children’s Hospital of Philadelphia (CHOP), we begin with establishing whether there is a treatable cause for the empty sella. We also test for any consequences (e.g., hormone deficiencies) that require specific therapy.

Many people with primary empty sella syndrome require no specific treatment, once the diagnosis is established.

If pituitary function is found to be abnormal, medical therapy may be used to address hormone deficiencies.

Outlook

Empty sella syndrome causes no health problems for many people who have the condition. When hormone production is affected, this can be treated with hormone replacement therapy, enabling the patient to lead a normal, healthy life.

Follow-up care

When the condition causes no symptoms and tests find no abnormal levels of hormone production, occasional follow-up tests are recommended to see that hormone production remains at normal levels.

When medication is needed to supplement hormone production, periodic follow-up tests are needed to ensure that the treatment continues to work effectively. Dosage levels and the combination of medications may need to be adjusted over time.

Why choose CHOP?

The Neuroendocrine Center at Children’s Hospital of Philadelphia offers families a coordinated and multidisciplinary approach to treatment for neuroendocrine disorders. Our team combines the expertise of pediatric endocrinologists, neuro-oncologists, neuro-surgeons, neuro-ophthalmologists, neuro-radiologists and pathologists.

All our team members have vast experience in the treatment of complex neuroendocrine conditions.