Introduction

Empty Sella syndrome (ESS) is a rare condition where your pituitary gland (a pea-sized organ at the base of your brain) becomes flattened.¹ About 1 in 6 people with this condition may have trouble seeing. This usually happens because the nerves that send pictures from your eyes to your brain can sink into the space around the flattened pituitary gland. 

In this article, you will learn:

• How ESS can affect your eyesight
• The most common visual problems people experience
• How doctors diagnose and treat the condition

What is Empty Sella Syndrome (ESS)?

Empty Sella syndrome (ESS) is a rare condition where your pituitary gland (a pea-sized organ at the base of your brain) becomes flattened. This pea-sized structure, described as the “master gland”, helps control many important functions in your body by releasing chemicals called hormones into your blood. Growth hormone helps your bones and muscles grow when you’re a child. Thyroid-stimulating hormone keeps your energy levels steady. Other hormones help your body handle stress, stay hydrated, and support fertility. Together, they keep your body working properly.¹

In the normal human skull, the pituitary gland sits in a small hole called the sella turcica, which means “Turkish saddle” in Latin, and it fills this space. We say someone has Empty Sella Syndrome (ESS) when this hole looks empty on scans. Instead of the pituitary gland, the space is filled with cerebrospinal fluid (CSF), the liquid that surrounds and protects the brain and spinal cord.

Brief explanation of how the condition develops: In empty sella syndrome, CSF pushes into the space where your pituitary gland sits, putting pressure on it and causing it to shrink or flatten. There are 2 types;

Primary empty sella syndrome happens when one of the protective layers around the brain bulges down into this space and presses on the gland. This occurs mostly in women who are middle-aged and those whose blood pressure is high.¹

Secondary empty sella syndrome occurs when the pituitary gland is damaged, resulting in a space. This can happen because of:

• A tumor
• Radiation treatment
• Surgery near the gland
• An injury to the head²

How empty sella syndrome affects vision

Normal setup

• The sella turcica is the small “seat” in the skull where the pituitary gland sits
• Just above it lies the arachnoid membrane (one of the coverings of the brain) and the third ventricle (a fluid-filled cavity in the brain)
• Nearby are the optic nerves/chiasm (the eye “wires”)

ESS can affect seeing in various ways³

• Optic chiasm compression: In ESS, your pituitary gland, which should normally fill your sella turcica, is flattened. This creates space around it, which is then filled with CSF. The optic chiasm is the area where the wires that carry images from both of your eyes come together before going to your brain. This X-shaped area lies just above the sella. It may be pushed into the empty sella by structures above, causing the wires to be stretched or compressed against the bony edges of the sella (remember the sella is part of the skull). This can make parts of your vision disappear, most commonly, bitemporal hemianopia, which is the loss of the outer half of vision in both of your eyes³
• Damage to the blood vessels of the optic chiasm: Because the sella is empty, the optic chiasm can sag into it. As it sags, the small blood vessels supplying the optic chiasm get stretched or pinched, which reduces the blood flow to your optic chiasm. This causes part of your optic chiasm to die off, which may make vision blurry or affect the colour and sharpness of vision³
• High pressure inside the skull: In primary ESS caused by high pressure inside your skull, the pressure is transmitted to your optic nerves, which are wires directly at the back of your eyes. It compresses them and reduces their blood flow, which damages them. Over time, this leads to optic atrophy, which is permanent damage to your optic nerves, causing vision loss⁴
• Adhesions in the sella after surgery: After surgery near the pituitary, scar tissue can form. This scar tissue pulls on the brain’s covering (arachnoid), dragging the fluid cavity above it (third ventricle) down into the pituitary space. That downward pull can squash the eye nerves and cause vision loss⁴

Common visual problems seen in ESS

Blurred vision

Loss of a section/part of what you normally see (visual field). It could be a patchy loss, or a loss in one-fourth of the visual field called quadrantanopia.

Reduced sharpness of vision (visual acuity)

• Sudden and temporary loss of vision: a few seconds of blacking out of vision
• Seeing double: if the wires controlling the movement of the eyes are affected
• Papilledema: swelling of a point at the back of the eye, which may be seen on an eye exam
• Optic atrophy, which means shrinking of the optic nerve, may occur due to pressure on the nerve for long periods or reduced blood reaching the nerve

These problems can lead to loss of vision, which may be temporary or, rarely, permanent.

Other symptoms that may accompany vision changes

Other symptoms are more common in ESS than vision changes. The most common symptom people with ESS have is headaches that don't go away. They may also have high blood pressure. Because ESS affects the pituitary gland, which is the “master gland” that controls many hormones, it can lead to changes in your hormone levels. The symptoms you may have depend on which particular hormones are affected. They include;

• Abnormal nipple discharge (galactorrhea)
• Men may experience difficulty with erections
• Women may experience irregular or missed periods
• Decreased libido
• Feeling dizzy or fainting
• Excessive thirst
• Urinating too much
• Fatigue
• Weight gain

In rare cases, people with ESS may also develop:

• High pressure inside the skull (sometimes called benign intracranial hypertension)
• A CSF leak from the nose (CSF rhinorrhea)

It’s also important to know that having an “empty sella” on a brain scan doesn’t always mean you’ll have symptoms. In fact, most people with an empty sella never develop any problems and may not even realise they have it.⁵

How doctors diagnose it

If your doctor suspects you have ESS, your medical history will be taken, you will be examined, and some tests /scans will be requested, which may include;

Eye tests

Visual acuity: where you read letters on a chart, typically sitting at a distance to check how sharp you can see.

• Visual field (Side vision test): where you press a button or look at lights to check if any part of your side vision is lost
• Fundoscopy (eye check with a special light): the doctor uses a special instrument to check the back of your eye
• Eye scan (OCT): which takes detailed pictures of the nerve and retina at the back of your eye

Additionally, brain CT (computed tomography), MRI (magnetic resonance imaging), and blood tests to evaluate hormone levels are conducted.

Management and treatment options

• For people who don't show any symptoms, the doctor may give appointments for regular check-ups and tests every 2 to 3 years
• Treatment for high pressure in the skull: This includes losing weight for those who are obese and reducing the amount of salt in their food. The doctor may prescribe drugs like acetazolamide and, in rare cases, recommend surgery to put in a small tube that helps drain the fluid in the brain
• Medications to treat abnormal hormone levels: It could be giving hormones in the form of drugs to increase the low levels in the blood or lowering excessively produced hormones

Surgery

In rare cases, when vision becomes really bad. It may include:

• Chiasmopexy: It lifts the optic chiasm back to its normal place, relieving pressure on it³
• Relieving pressure around the optic nerve behind the eye⁴
• CSF leak repair if present, to prevent infection

Prognosis

The outlook is generally good. It usually does not affect how long a person is expected to live, and most people live without major problems. Vision can be preserved if visual problems are detected and treated early. The prognosis depends on which hormones are affected. Medicines can help correct hormone problems caused by ESS. You might need to stay on treatment long-term, maybe for life. Those who have no symptoms and normal hormone levels usually don't get worse.

Summary

Syndrome (ESS) is a rare condition in which the pituitary gland, a small pea-sized organ at the base of the brain, becomes flattened. It can occur in two forms: primary ESS and secondary ESS. About 1 in 6 people with this condition may develop vision problems. These issues can arise when the optic nerves—the “wires” that carry visual signals from the eyes to the brain-sink into the space around the flattened gland or when their blood supply is damaged, causing parts of the nerves to stop working. As a result, people may experience reduced visual clarity, loss of side vision, or brief episodes in which their vision goes dark. Doctors diagnose ESS using brain scans and eye exams. Treatment may involve weight loss, medications that lower pressure in the brain, hormone replacement, or surgery. Overall, the outlook is good, and most people live normal lives without major complications or changes in life expectancy.