Empty Sella Syndrome

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:

• Fatigue
• 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

Stage: Recruiting

Contact: Prashant Chittiboina, MD

National Institutes of Health Clinical Center

Bethesda, MD 

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

Design:

Participants will be screened with:

Medical history

Physical exam

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

Stage: Recruiting

Principal investigator: Constantine A Stratakis, MD

National Institutes of Health Clinical Center

Bethesda, MD 

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

Stage: Recruiting

Principal investigator:  Nathan E Simmons, MD  

Dartmouth Hitchcock Medical Center

Lebanon, NH

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.