What is Empty Sella Syndrome?
Empty sella, also known as arachnoidocele, is a condition discovered through imaging tests, where a certain part of the brain appears empty due to the presence of cerebral spinal fluid (CSF). This fluid flows into the sella turcica (a kind of bone cavity in the skull) and causes the pituitary gland (a small hormone-regulating organ) within it to become compressed and flat. It also stretches the pituitary stalk, which connects the pituitary gland to the brain.
In the past, this was accidentally encountered during brain imaging using CT and MRI scans. Nowadays, because of improvements in radiologic technology, the occurrence of empty sella has increased. While empty sella was previously thought to be an incidental finding without any significant clinical impact, recent evidence suggests that patients might experience associated symptoms, collectively known as ESS, more often than we used to think. Because of this, most experts now believe that it’s worth having a thorough medical check-up if someone is found to have an empty sella. Even patients without symptoms could benefit from a reevaluation. If a patient does have symptoms of ESS, the treatment can range from drug therapy (like growth hormone replacement) to surgery.
Considering that empty sella is being recognized more frequently and can be associated with more symptoms than we initially thought, it’s important for doctors to be familiar with this condition and its possible outcomes. In managing and diagnosing ESS, it’s crucial for various healthcare professionals, including eye doctors, neurologists, and hormone specialists, to work together to improve the patient’s health.
What Causes Empty Sella Syndrome?
“Empty sella” is a term doctors use to describe a specific finding on medical scans, often discovered by accident. This finding results from a weakness in the diaphragm sellae (this is a small, thin piece of tissue that separates the pituitary gland from the brain), causing cerebrospinal fluid (CSF, which is the fluid around your brain and spinal cord), to push into a space in the skull called the sella turcica.
When this situation is linked with symptoms like headaches and changes in vision, doctors call it empty sella syndrome (ESS). This syndrome is separated into two types: primary empty sella (PES) and secondary empty sella (SES). PES occurs without any clear cause, while SES happens because of a known reason like a brain injury or damage to the pituitary gland during childbirth.
There are also “complete” and “partial” versions of ESS. A “complete” ESS means that over half of the sella turcica is filled with CSF, while in “partial” ESS, less than half of the sella turcica is filled with CSF.
The cause of PES is largely unknown; though, there might be a genetic link for certain people who have defects in the diaphragm sellae. On the other hand, SES can be caused by surgery, injury, radiation treatment, bleeding, infection, or damage to the pituitary gland. It can occur at any point in a person’s life.
Risk Factors and Frequency for Empty Sella Syndrome
The ’empty sella’ condition was previously thought to be quite rare. However, because of improvements in medical imaging, more cases are being identified. Autopsies reveal this condition in about 6% to 20% of cases. It is also found in around 38% of patients who get brain imaging tests, especially MRI scans. This condition is seen more than five times more often in women, frequently in people who are overweight and in individuals who are between 40 to 60 years old. It’s also important to note that around 40% of people with ’empty sella’ also have hormone irregularities.
- The ’empty sella’ condition was once considered rare.
- Improvements in medical imaging have led to an increase in its diagnosis.
- It’s found in 6% to 20% of autopsy cases.
- Around 38% of patients who get brain scans, especially MRIs, are found to have this condition.
- It’s more than five times as common in women.
- It’s also more common in overweight individuals and those aged between 40 to 60.
- About 40% of ’empty sella’ patients also have hormone abnormalities.
Signs and Symptoms of Empty Sella Syndrome
An empty sella is a condition often discovered accidentally during scans for other concerns. Although it’s not clear how this condition is linked to visual, mental, or hormonal issues, patients diagnosed with empty sella frequently suffer from these conditions. For example, hormonal irregularities are present in about 40% of people with empty sella, and as many as 94% have high pressure within the skull. Due to this, experts suggest thorough assessment, even if the person isn’t showing any symptoms.
Regardless of the patient, medical professionals should always gather a full medical history and conduct a physical examination. Details about pregnancies, surgical operations, head traumas, or radiation therapies can help doctors understand potential causes for empty sella. Mostly, people with asymptomatic empty sella, meaning they don’t display symptoms, have normal medical history and physical exam findings since the hormone functions are usually unaffected. However, those with symptomatic empty sella may experience headaches, vision troubles, dizziness, fainting, menstrual irregularities, lactation, erectile dysfunction, excessive thirst, and urination. Some rare cases report issues like brain fluid leakage from the nose or unusual conditions like low sodium levels in the blood and cognitive impairment.
Physical examination for empty sella can either be normal or show signs like high blood pressure, hormonal imbalances manifesting as enlarged male breasts, bodily changes from excessive growth hormone, and signs of decreased sex hormones. Additionally, they often carry more weight than normal, resulting in conditions like sleep apnea and high body mass index.
Testing for Empty Sella Syndrome
If someone has an “empty sella,” which means their pituitary gland (a small gland located at the base of the brain) appears absent or flattened on an image from a CT or MRI scan, doctors often find that a certain type of fluid (known as CSF) fills up this space in the brain. The total volume of the pituitary, usually less than 611.21 mm³, appears to have shrunk and the bone structures around the gland look broader than usual.
There are two types of this condition – partial and complete. If less than half of the pituitary gland space is filled with CSF, it’s called a partial empty sella, but if CSF fills more than half of it, the condition is known as a complete empty sella.
If you’re diagnosed with an empty sella, it’s important to have your pituitary gland’s function checked, even though it might look unusual on the scan. The pituitary gland regulates several key hormones in the body, so to make sure it’s working properly, your doctor might recommend some blood tests. These tests will check your hormone levels, such as your body’s production of the growth hormone, prolactin, etc.
In some people with empty sella, these hormone levels may be lower than normal. Impaired growth hormone and prolactin levels are common findings. About 10-17% show higher than normal prolactin levels, due to either a small pituitary tumor or functional hyperprolactinemia, a condition where the pituitary gland produces too much prolactin.
When your doctor tests your pituitary gland function, they’ll likely look at:
– Your body’s production of cortisol, a hormone that helps control your body’s use of fats, proteins, and carbohydrates. Low levels can point to an adrenal insufficiency.
– Your thyroid-stimulating hormone and free thyroxine (T4) levels. These hormones both play an essential role in controlling the thyroid gland.
– Your reproductive hormones, such as follicle-stimulating hormone, luteinizing hormone, and estradiol. These hormones regulate processes like menstrual cycles in women and testosterone levels in men.
– Your prolactin level, which regulates reproductive health.
– Your growth hormone level. This hormone is crucial for growth in children and helps maintain tissues and organs in adults.
– Your antidiuretic hormone level, which balances your body’s water level.
Treatment Options for Empty Sella Syndrome
Though it’s still a developing field with no set guidelines, the treatment of ESS or Empty Sella Syndrome usually focuses on addressing symptoms and managing any hormonal imbalances. This syndrome impacts the pituitary gland, an important part of our endocrine system, which produces numerous hormones.
If a patient’s hormone levels are found to be imbalanced, their treatment is adjusted accordingly. For example, if the pituitary gland isn’t producing enough hormones (a condition known as hypopituitarism), doctors may recommend a treatment plan that includes cortisol replacement therapy. If the patient’s body is producing too much prolactin (a condition known as hyperprolactinemia), they might benefit from drugs that increase dopamine – a chemical that helps regulate prolactin levels.
If ESS has caused pressure to build up in the brain – a condition known as intracranial hypertension – doctors may recommend medication or possibly even surgery to relieve this pressure. They may suggest medications that reduces fluid production in the brain, or could consider surgically implanting a device called a ventriculoperitoneal shunt, which helps drain excess fluid from the brain. Which treatment is best depends upon a number of patient-specific factors, such as the severity of symptoms and overall health.
Regular check-ups are a key part of managing ESS. These allow your medical team to monitor any associated conditions and adjust your treatment as needed.
If a patient has ESS but does not have any symptoms or hormone abnormalities, the condition may not pose significant health risks. There is limited evidence that ESS will worsen in such cases. Regular clinical assessments every 2 to 3 years, or as deemed necessary by the patient’s healthcare provider, are generally recommended to monitor the patient’s condition over time.
What else can Empty Sella Syndrome be?
When a doctor notices an “empty sella” during an imaging scan, it means that the sella turcica (a small area in the skull where the pituitary gland resides) is empty. The condition named after this finding could be due to a number of underlying causes, including:
- Pituitary gland tumors
- A condition known as idiopathic pseudotumor cerebri that causes increased pressure in the brain
- A blood clot within the skull, known as intracranial thrombosis
- Build-up of fluid in the brain, or hydrocephalus
- Cysts or abnormal growths in the pituitary gland
- Birth defects affecting the pituitary gland
- Type of pituitary tumors that produce too much of the hormone prolactin, called cystic prolactinomas
- A type of brain tumor known as craniopharyngioma
- A type of brain cyst known as an arachnoid cyst
It’s important to clarify that each of these conditions has distinct features and may require different approaches to treatment.
What to expect with Empty Sella Syndrome
Ectopic endocrine syndromes (EES), usually harmless, don’t typically affect how long a person lives. The outcome generally hinges on the specific hormonal imbalance, if there is one. Current research shows that individuals who don’t show symptoms and don’t have hormone abnormalities usually see little progress in the condition.
Possible Complications When Diagnosed with Empty Sella Syndrome
Potential complications from the medical condition known as “empty sella” can involve problems with the pituitary gland, specifically underactive or overactive hormone production. There can be heightened risk in patients who have “primary empty sella”, such as in Cushing’s disease. If these patients have also suffered disruption of the tissue connecting the pituitary gland to the brain (the pituitary stalk) seen on X-rays, they may have poorer results after undergoing pituitary surgery.
Common Complications:
- Partial or complete lack of pituitary gland activity
- Overactive hormone production
- Worse outcomes from pituitary surgery, especially with Cushing’s disease
Preventing Empty Sella Syndrome
Patients cannot actively prevent this condition, but it is essential for them to realize the importance of checking their hormone levels when first diagnosed. Additionally, individuals who show no symptoms or signs of hormone irregularities should know that there’s limited evidence of this condition getting significantly worse. However, should they feel any changes or have concerns, they should seek regular medical check-ups and tests if their doctor deems it necessary.
