What is Empty Sella Syndrome?
Empty Sella Syndrome (ESS) is a disorder that affects the sella turcica, a bony structure in the center of the skull beneath the brain. The sella forms a small cavity that encloses the pituitary gland, an organ that regulates the production of many different hormones in different parts of the body. The pituitary gland normally fills the sella, but in ESS, the gland is flattened against the wall of the cavity. In imaging scans, the sella appears to be empty.
Since Empty Sella Syndrome is only detectable by a brain imaging scan, scientists are not sure how common the disorder is. Some studies suggest that it could affect up to 12% of the population, but clinical observations have suggested that it is more common, affecting 25% of the population or more.
In most cases, Empty Sella Syndrome (ESS) causes no symptoms or complications, and no treatment is required. When the disorder does cause symptoms, the symptoms are usually treatable and not life-threatening.
Empty Sella Syndrome (ESS) is divided into two categories, primary Empty Sella Syndrome and secondary Empty Sella Syndrome.
Primary Empty Sella Syndrome
Primary Empty Sella Syndrome occurs when cerebrospinal fluid (CSF) enters the sella and pushes the pituitary gland against the side of the sella cavity. CSF is normally kept out of the sella by a membrane, but sometimes the fluid infiltrates the cavity, taking up space normally occupied by the pituitary gland.
Secondary Empty Sella Syndrome
Secondary Empty Sella Syndrome is a result of damage to the pituitary gland that reduces the size of the gland. This can happen after a head injury, surgery, or radiation treatment for a pituitary tumor.
Symptoms of Empty Sella Syndrome
In most cases, Empty Sella Syndrome does not cause symptoms. The pituitary gland continues to function normally despite being displaced from its usual position. In some cases, however, the function of the gland is impaired, and symptoms of hormone imbalances occur. These symptoms can include:
- Decreased sex drive
- Impotence in men
- Irregular menstrual periods in women
Some people experience symptoms that might be related to higher than normal CSF pressure in the skull. These symptoms can include:
- Vision disturbances
- High blood pressure
In rare cases, Empty Sella Syndrome might cause damage to the floor of the sella itself. When this happens, cerebrospinal fluid might leak into the nose. This complication is more common in secondary Empty Sella Syndrome, and it may require surgery to correct.
What Causes Empty Sella Syndrome?
The cause of primary Empty Sella Syndrome is not well understood. The condition occurs when the membrane covering the sella turcica (the diaphragma sellae) allows cerebrospinal fluid to enter the sella. Sometimes, a patient could have a defect in the membrane from birth, and the defect could be responsible for the failure of the membrane to keep fluid out of the sella. In some cases, however, there is no congenital defect in the membrane that is an obvious possible cause.
Empty Sella Syndrome is often associated with higher than normal pressure in the cerebrospinal fluid throughout the skull, a condition called idiopathic intracranial hypertension (IIH). About 70% of people with IIH have Empty Sella Syndrome, but IIH is rare, and most people with Empty Sella Syndrome don’t have IIH. This suggests that IIH is unlikely to be the cause of Empty Sella Syndrome.
The causes of secondary Empty Sella Syndrome are more clear because the disorder, by definition, follows a trauma, injury, or other damage to the area of the sella turcica. Secondary ESS is more likely to cause easily observable symptoms such as hormone imbalances and vision disturbances.
Is Empty Sella Syndrome Hereditary?
Empty Sella Syndrome is not inherited. Research has found no evidence that the disorder runs in families, and there is no known connection between Empty Sella Syndrome and any genes or inherited factors.
How Is Empty Sella Syndrome Detected?
Because it often doesn’t produce symptoms, Empty Sella Syndrome probably escapes detection in most cases. Often, the disorder is detected incidentally when the patient undergoes a brain imaging exam for some other reason.
Empty Sella Syndrome can sometimes cause abnormally low levels of various hormones throughout the body, a condition called hypopituitarism. The symptoms of hypopituitarism sometimes escape notice because they can be relatively subtle and develop gradually over a long period of time.
You should consult a doctor if you experience signs of pituitary-related hormone deficiency, including:
- Muscle weakness
- Unexplained weight gain
- Irregular menstrual periods
- Loss of facial or pubic hair
- Decrease in sex drive
- Erectile dysfunction
- Difficulty with the production of breast milk while nursing
Sometimes hypopituitarism can be caused by an unrelated disorder called pituitary apoplexy. This condition is caused by bleeding into the pituitary gland and is life-threatening. Seek immediate emergency care if you experience symptoms of hypopituitarism that come on suddenly or are accompanied by a severe headache, visual disturbances, confusion, or low blood pressure.
How Is Empty Sella Syndrome Diagnosed?
If you have symptoms characteristic of Empty Sella Syndrome, your doctor will likely conduct a physical exam and take a medical history to rule out other potential causes of the symptoms. Empty Sella Syndrome can be definitively diagnosed using imaging exams such as computerized tomography (CT) or magnetic resonance imaging (MRI) scans.
If you are showing signs of hypopituitarism, your doctor will probably order blood tests that measure the level of different hormones in your bloodstream. If the tests indicate that hormone levels are too low or too high, it might be an indication that your pituitary gland is not functioning properly. In most cases of Empty Sella Syndrome, the pituitary function is normal.
PLEASE CONSULT A PHYSICIAN FOR MORE INFORMATION.
How Is Empty Sella Syndrome Treated?
When ESS is detected by an imaging scan but no symptoms are present, no treatment is usually recommended. If there are symptoms, they are treated individually. In the case of secondary ESS caused by an underlying injury or condition, the underlying cause of the disorder is the target of treatment.
In the event that treatment is recommended, potential treatment approaches can include:
- Hormone replacement therapies are called for when specific hormone deficiencies are identified.
- Surgery may be required if the cerebrospinal fluid is leaking through the nose, a condition called CSF rhinorrhea.
How Does Empty Sella Syndrome Progress?
As long as the function of the pituitary gland isn’t impaired, ESS is unlikely to cause serious symptoms or long-term complications. Usually, and especially in the case of primary ESS, it’s very unlikely that the disorder will cause significant health problems.
If the pituitary function is impaired, and the dysfunction goes untreated, health issues can emerge. Potential complications include:
- Growth problems or short stature in children with growth hormone (GH) deficiency
- Delayed puberty in children with luteinizing hormone (LH) or follicle-stimulating hormone (FSH) deficiency
- Impotence or infertility in men with luteinizing hormone (LH) or follicle-stimulating hormone (FSH) deficiency
- Infertility in women with luteinizing hormone (LH) or follicle-stimulating hormone (FSH) deficiency
- Weight gain in people with thyroid-stimulating hormone (TSH) deficiency
- Insufficient breast milk production in women with prolactin deficiency
- Breast milk production when not nursing or breast development in men with high prolactin levels
How Is Empty Sella Syndrome Prevented?
The cause of primary Empty Sella Syndrome remains unknown, so there is no definitive way to prevent the disorder. ESS is most common in middle-aged women who are obese and have high blood pressure. It’s not clear whether these characteristics represent risk factors for developing the disorder, but certain lifestyle changes are a good idea in any case:
- Weight loss if you’re obese
- Avoidance of rapid weight gain
- Regular exercise
- Treatment of high blood pressure
Empty Sella Syndrome Caregiver Tips
Keep these tips in mind when your loved one is suffering from symptoms associated with ESS:
- Take the disorder seriously. ESS usually doesn’t cause health problems, but when it does, they are real problems. Chronic headaches are the most common symptom of ESS, and chronic pain can be exhausting and debilitating. Show your loved one that you are empathetic and that you’ll do what you can to help.
- Be supportive of treatment and lifestyle changes. Help your loved one stick with any prescribed treatment plan and any lifestyle changes that their healthcare providers recommend.
Many people with ESS also suffer from other brain and mental health-related issues, a condition called co-morbidity. Here are a few of the disorders commonly associated with ESS:
- People with ESS may be at increased risk of migraines.
- ESS is sometimes associated with intracranial hypertension (IIH), which is itself associated with other brain-related conditions.
- Eating disorders have been associated with IIH.
- Some studies have linked higher rates of anxiety with IIH.
- People with IIH are at increased risk of depression.
Empty Sella Syndrome Brain Science
Empty Sella Syndrome can have far-reaching effects throughout the body because of the pituitary gland’s important role in regulating the hormone production of many other glands. The pituitary gland is directly connected to a part of the brain called the hypothalamus. This part of the brain is able to detect the levels of hormones produced by other glands. When necessary, the hypothalamus triggers the pituitary gland to release its own hormones, which stimulate other glands and organs.
Hormones produced by the pituitary gland include:
- Growth hormone (GH). This hormone promotes muscle and bone growth and fat reduction.
- Thyroid-stimulating hormone TSH). This hormone causes the thyroid to produce hormones that control metabolism.
- Adrenocorticotropic hormone ( ACTH). This hormone causes the adrenal glands to produce hormones that control metabolism, the immune system, blood pressure, stress response, and other body functions.
- Follicle-stimulating hormone (FSH) and luteinizing hormone (LH). These hormones stimulate the production of reproductive cells and the sex hormones testosterone and estrogen.
- Prolactin. This hormone stimulates the production of breast milk in the mammary glands.
Empty Sella Syndrome Research
Title: Feasibility of Endosphenoidal Coil Placement for Imaging of the Sella During Transsphenoidal Surgery
Contact: Prashant Chittiboina, MD
National Institutes of Health Clinical Center
Background: Pituitary tumors can cause problems by secreting hormones in the body. They can also problems by growing large and pushing on organs near the pituitary gland. The best treatment for such tumors is to remove them by surgery. But that may be sometimes difficult. Some tumors may be too small to see. Some other tumors are maybe so large that portions may be left behind during surgery. The endosphenoidal coil (ESC) is a new magnetic resonance imaging (MRI) device. It fits in a small space made during surgery near the pituitary. Researchers want to see if it helps transmit MRI signals during surgery to make better images of the pituitary gland and tumors.
Objective: To test the safety of using a new coil device to improve MRI imaging of pituitary tumors during surgery.
Eligibility: Adults 18-65 years old who are having pituitary tumor surgery at NIH
Participants will be screened with:
Review of prior brain scans
Blood and pregnancy tests
All participants will have MRI of the pituitary gland. They will lie on a table that slides into a metal cylinder in a strong magnetic field. They will lie still and get earplugs for loud sounds. A dye will be inserted into an arm vein by a needle.
Participants will stay in the hospital for about 1 week. They will repeat screening tests.
Participants will have standard pituitary surgery. They will get medicine to go to sleep. The surgeon will create a path to the pituitary gland from under the lip.
During surgery, the ESC will be placed through the path to near the pituitary. Then an MRI will be done during surgery.
Then the ESC will be removed and standard surgery will continue.
Participants will get standard post-operative care under another protocol.
Title: An Investigation of Pituitary Tumors and Related Hypothalmic Disorders
Principal investigator: Constantine A Stratakis, MD
National Institutes of Health Clinical Center
There is a variety of tumors affecting the pituitary gland in childhood; some of these tumors (eg craniopharyngioma) are included among the most common central nervous system tumors in childhood. The gene(s) involved in the pathogenesis of these tumors are largely not known; their possible association with other developmental defects or inheritance pattern(s) has not been investigated. The present study serves as a (i) screening/training, and, (ii) a research protocol.
As a screening and training study, this protocol allows our Institute to admit children with tumors of the hypothalamic-pituitary unit to the pediatric endocrine clinics and wards of the NIH Clinical Center for the purposes of
(i)training our fellows and students in the identification of genetic defects associated with pituitary tumor formation, and
(ii)teaching our fellows and students the recognition, management, and complications of pituitary tumors
As a research study, this protocol aims at
(i)developing new clinical studies for the recognition and therapy of pituitary tumors; as an example, two new studies have emerged within the context of this protocol: (a) investigation of a new research magnetic resonance imaging (MRI) tool and its usefulness in the identification of pituitary tumors, and (b) investigation of the psychological effects of cortisol secretion in pediatric patients with Cushing disease. Continuation of this protocol will eventually lead to new, separate protocols that will address all aspects of diagnosis of pituitary tumors and their therapy in childhood.
(ii)Identifying the genetic components of pituitary oncogenesis; those will be investigated by (a) studying the inheritance pattern of pituitary tumors in childhood and their possible association with other conditions in the families of the patients, and (ii) collecting tumor tissues and examining their molecular genetics. As with the clinical studies, the present protocol may help generate ideas for future studies on the treatment and clinical follow up of pediatric patients with tumors of the pituitary gland and, thus, lead to the development of better therapeutic regimens for these neoplasms.
Title: Utilization of iMRI for Transsphenoidal Resection of Pituitary Macroadenomas
Principal investigator: Nathan E Simmons, MD
Dartmouth Hitchcock Medical Center
The investigators are studying the utility of intraoperative magnetic resonance imaging (iMRI) during transsphenoidal pituitary surgery for large macroadenomas by randomizing patients to either an intra-operative MRI after resection or no intra-operative MRI. The investigators will then count the number of gross total resection in each group of patients and also the complications related to surgery.