What is empty sella syndrome?
Empty sella syndrome is a rare disorder related to a part of the skull called the sella turcica. The sella turcica is an indentation in the sphenoid bone at the base of your skull that holds the pituitary gland.
If you have empty sella syndrome, your sella turcica is not actually empty. In fact, it means your sella turcica is either partially or totally filled with cerebrospinal fluid (CSF). People with empty sella syndrome also have smaller pituitary glands. In some cases, the pituitary glands don’t even show up on imaging tests.
When empty sella syndrome is caused by an underlying condition, it’s called secondary empty sella syndrome. When there’s no known cause, it’s called primary empty sella syndrome.
What are the symptoms?
Empty sella syndrome usually doesn’t have any symptoms. However, if you have secondary empty sella syndrome, you may have symptoms related to the condition that’s causing it.
Many people with empty sella syndrome also have chronic headaches. Doctors aren’t sure if this is related to empty sella syndrome or to high blood pressure, which many people with empty sella syndrome also have.
In rare cases, empty sella syndrome is associated with pressure building up in the skull, which can lead to:
- spinal fluid leaking from the nose
- swelling of the optic nerve inside the eye
- vision problems
What are the causes?
Primary empty sella syndrome
The exact cause of primary empty sella syndrome isn’t clear. It may be related to a birth defect in the diaphragma sellae, a membrane that covers the sella turcica. Some people are born with a small tear in the diaphragma sellae, which can cause CSF to leak into the sella turcica. Doctors aren’t sure whether this is a direct cause of empty sella syndrome or simply a risk factor.
According to the National Organization for Rare Disorders, empty sella syndrome affects about four times as many women as it does men. Most women with empty sella syndrome tend to be middle-aged, obese, and have high blood pressure. However, most cases of empty sella syndrome go undiagnosed due to their lack of symptoms, so it’s hard to say whether gender, obesity, age, or blood pressure are true risk factors.
Secondary empty sella syndrome
A number of things can cause secondary empty sella syndrome, including:
- head trauma
- pituitary tumors
- radiation therapy or surgery in the area of the pituitary gland
- conditions related to the brain or pituitary gland, such as Sheehan syndrome, intracranial hypertension, neurosarcoidosis, or hypophysitis
How is it diagnosed?
Empty sella syndrome is hard to diagnose because it usually doesn’t produce any symptoms. If your doctor suspects you might have it, they’ll start with a physical exam and a review of your medical history. They’ll also probably order CT scans or MRI scans.
These scans will help your doctor determine whether you have partial or total empty sella syndrome. Partial empty sella syndrome means your sella is less than half full of CSF, and your pituitary gland is 3 to 7 millimeters (mm) thick. Total empty sella syndrome means more than half of your sella is filled with CSF, and your pituitary gland is 2 mm thick or less.
How is it treated?
Empty sella syndrome usually doesn’t require treatment unless it’s producing symptoms. Depending on your symptoms, you may need:
- surgery to prevent CSF from leaking out of your nose
- medication, such as ibuprofen (Advil, Motrin), for headache relief
If you have secondary empty sella syndrome due to an underlying condition, your doctor will focus on treating that condition or managing its symptoms.
What’s the outlook
On its own, empty sella syndrome usually doesn’t have any symptoms or negative effects on your overall health. If you have secondary empty sella syndrome, work with your doctor to diagnose and treat the underlying cause.