Exophthalmos

What is Exophthalmos?

Exophthalmos, also known as proptosis, is a condition where one or both eyes protrude or bulge out significantly. The name comes from a Greek word that literally means ‘bulging eyes.’ This unusual protrusion happens when there is an increase in the amount of tissue or other substance inside the orbit, the bony part that contains the eye.

The exact symptoms that come with exophthalmos can depend on why it’s happening. Some people may have additional symptoms throughout their body. The vision can also be affected if the optic nerve, which sends visual information to the brain, gets squished due to the increased pressure inside the orbit.

What Causes Exophthalmos?

In adults, a condition often related to the thyroid called Graves-related ophthalmopathy, is usually the main reason for unilateral and bilateral exophthalmos, a term used for eyes that bulge out of the eye sockets.

In children, a condition known as orbital cellulitis is the most common cause. If both eyes are bulging (bilateral exophthalmos), it’s often likely due to diseases such as neuroblastoma and leukemia.

Generally, there are four main types of causes for this eye bulging:

1. Spreading of inflammation into the eye socket. This can occur in conditions such as thyroid-related eye disease, orbital cellulitis (an infection near the eye), sarcoidosis (inflammation that produces tiny lumps of cells in organs), granulomatosis with polyangiitis (a rare disease causing inflammation of blood vessels), and IgG4-related disease (an immune system disorder).

2. New growths invading the eye socket such as benign or malignant tumors. These include conditions like capillary haemangioma (a strawberry-like birthmark), neuroblastoma, neurofibromatosis (a genetic disorder that causes tumors to form on nerve tissue), leukemia, lymphoma, mucocele (a swelling in the mouth), pseudotumors, and secondary metastatic deposits (cancer that has spread from another part of the body).

3. Disruptions in the flow of blood back from the eye, for example, due to diseases like orbital varices (veins in the eye socket that become enlarged and twisted), carotid-cavernous fistula (an abnormal connection between a large artery and a large vein in the face or neck), and cavernous sinus thrombosis (a blood clot in part of the brain).

4. Foreign objects forced into the eye socket, typically due to trauma or injury.

Risk Factors and Frequency for Exophthalmos

The occurrence of exophthalmos, a condition where the eyes bulge out, can vary based on the underlying cause. For unilateral exophthalmos, when only one eye is affected, less than a third of patients will show signs of overactive thyroid. Most instances of bilateral exophthalmos, where both eyes are affected, historically result from hormone-related disorders. On average, the eye protrusion measures about 16 mm, assessed using a tool called an exophthalmometer. The extent of exophthalmos may differ between genders and races.

The rate of exophthalmos varies depending on the cause.
For unilateral exophthalmos (one eye affected), less than a third of patients have an overactive thyroid.
In the past, 90% of bilateral exophthalmos cases (both eyes affected) were due to hormone-related disorders.
The average eye protrusion, as measured with an exophthalmometer, is about 16 mm.
The severity of exophthalmos can differ between men and women, and among different racial groups.

Signs and Symptoms of Exophthalmos

The symptoms of exophthalmos, or bulging eyes, can differ based on what’s causing the condition. Some common symptoms include:

Bulging eyes that can be measured with a device called an exophthalmometer.
Swelling of the eyelid or around the eye, which could be on one or both sides. This can be associated with a reaction in the lining of the eye, or an infection called orbital cellulitis.
Double vision, which could be due to muscles around the eyes being affected by inflammation or compression from a growing tumor.
Redness in the eyes, which can worsen with bulging eyes due to dilation. In severe cases, this can lead to exposure keratopathy, a condition resulting from the upper eyelid not fully covering the cornea.
Muscle weakness in the eyes, commonly seen in infectious, inflammatory conditions, or aggressive tumors.
Decreased sharpness of vision.

A thorough medical history can help identify the cause of these symptoms. For instance, symptoms such as heat intolerance, weight loss, changes in bowel habits, and palpitations may indicate a thyroid condition. A history of trauma or symptoms like weight loss could point towards cyst growth or a tumor. The speed of symptom onset can also provide clues – for instance, quick onset could indicate inflammatory disease, malignant tumors, or a condition called carotid-cavernous fistula, while gradual onset could suggest a less severe condition. The presence of pain often points towards an infection. Temporary bulging of the eyes triggered by a specific type of breathing called the Valsalva maneuver could be a sign of blood vessels in the eye being affected.

An examination can help identify the cause. This should include checking for diseases such as Graves’ disease, leukemia, or tumors in the torso area, or any signs that could suggest cancer. A full eye examination is also crucial, including checking eye movement, sharpness of vision, pupil response, and eye field. Pressure inside the eye, and checks of the front of the eye and the inner lining of the eye, called fundoscopy, should also be performed.

Bulging of the eyes can be quantified using an exophthalmometer, which measures the distance from the front of the eye to the middle of the front edge of the eye socket. Bulging eyes can be accompanied by other symptoms relevant to systemic causes. It’s important that the doctor is at eye level with the patient during the check. In cases of exophthalmos, the white of the eye below the colored part of the eye is often visible.

Testing for Exophthalmos

If you have symptoms that suggest a serious infection or a condition affecting your eyes or brain, your doctor will order a series of tests. One of the first steps will be a set of blood tests, which may include a complete blood count (CBC) to measure the number of different cells in your blood, thyroid function tests which can help see how well your thyroid gland is working, and auto-antibody tests to look for certain kinds of proteins that your body might make if it’s fighting an illness. Your doctor might also do some tests to see how well your kidneys are working and look for C-reactive protein, a substance produced by the liver in response to inflammation. If your doctor suspects a severe infection, like orbital cellulitis (a severe infection that affects the tissues around your eye), they may also take a sample from your nose or blood to check for harmful bacteria.

Your doctor will also likely order some imaging tests. These can include a computed tomography (CT) scan or magnetic resonance imaging (MRI), which are powerful tools that can create detailed pictures of the inside of your body. These tests are particularly good at identifying issues in the orbit (the bony socket that contains your eye) or cranium (the part of your skull that houses your brain), like serious infections, abnormal growths, or foreign objects. Another test that you may need is a positron emission tomography (PET) scan. This type of scan can help your doctor check for metastatic disease, which is a condition where cancer spreads from its original site to other parts of the body. Some of the cancers that frequently spread in this manner include leukemia, lymphoma, and secondary cancers.

It can sometimes be challenging for your doctor to firmly diagnose your condition based on symptoms and tests alone, because many different conditions can cause similar symptoms. In such cases, your doctor may need to perform a biopsy to get a definitive answer. This involves taking a small sample of tissue from the affected area to examine under a microscope.

If you’re experiencing proptosis or exophthalmos, which are fancy medical terms for when one or both of your eyes bulge out of its usual position in the eye socket, it could potentially be linked with other issues related to the position of your eyeball. This could include hyperglobus (when your eyeball is positioned too high in the socket), hypoglobus (when it’s positioned too low), esoglobus (when it’s positioned too close to your nose), or exoglobus (when it’s positioned too far from your nose).

Treatment Options for Exophthalmos

To manage exophthalmos, which is a condition where the eyeball protrudes from the socket, it’s important to treat the root cause in order to preserve eye function. This often involves close collaboration among eye specialists, primary care providers and endocrinologists, especially when the exophthalmos is related to thyroid disease or other secondary causes.

If you’re a smoker, quitting smoking is crucial to both prevent and slow the progression of thyroid eye disease, which can lead to exophthalmos.

While addressing the underlying cause of exophthalmos, there are various supportive treatments that can provide symptom relief. Patients dealing with dry eyes from the condition can use preservative-free eye lubricants or tape their eyelids. Since many mild cases often resolve within six months, these kinds of supportive treatments may be sufficient. Wearing sunglasses and protective eyewear is also recommended to help safeguard against light sensitivity and glare. If you’re experiencing diplopia, or double vision, this can often be managed with something called a Fresnel prism or with methods to temporarily block vision in one eye. In some cases, an injection of botulinum toxin can be used to correct upper eyelid retraction.

For more severe cases of thyroid-related exophthalmos, oral and intravenous corticosteroids may be prescribed to reduce swelling and ease congestion in the orbital area, which is the area around the eye. If the exophthalmos is due to inflammation or an autoimmune issue, this treatment can be particularly helpful. In instances where a tumor is causing the exophthalmos, chemotherapy agents might be used to help shrink the tumor.

When the eye protrusion is due to tissue overgrowth, a tumor, or cancerous disease, surgery may be necessary. Specific surgical procedures, like orbital decompression (which creates more space for the eye) and extraocular muscle repair (which fixes muscles controlling eye movement), have proven effective in preserving vision, particularly in severe cases or situations where medical therapy hasn’t worked. Some reports show that these surgical methods can improve visual function in up to 82% of cases.

When trying to diagnose a condition related to autoimmune diseases, inflammation, injuries, or cancer, it’s important for doctors to consider other conditions that present similar symptoms. These can include:

Conditions related to the thyroid, such as orbitopathy associated with Graves’ disease
Infections, like orbital and preseptal cellulitis
Abnormal blood vessel formations, such as carotid-cavernous fistula
Both harmless and harmful growths, such as capillary haemangioma, neuroblastoma, leukemia, lymphoma, mucocele, or pseudotumors, and also secondary spread of cancer from other parts of the body that can affect the orbit

Other less common conditions like Crouzon syndrome and Apert syndrome may also need to be considered. Additionally, injuries causing fractures around the eyes could lead to bleeding around the eyes and potentially bulging of the eyes.

What to expect with Exophthalmos

Figuring out the root cause of exophthalmos, a condition where the eyes bulge out of their sockets, is crucial for treatment. After identifying the cause, any associated swelling, pain, or redness usually gets better on its own within 2 to 3 months, although the timeline can vary from person to person.

Exophthalmos related to thyroid issues can take longer to resolve, and in some cases, may not return to its normal state. In fact, about 5% of these cases might end up with permanent double vision or continue to have lasting vision impairment.

Possible Complications When Diagnosed with Exophthalmos

The complications that generally occur are associated with the original health issue. For instance, if the cornea, which is the front surface of your eye, is exposed for a long time, it might become extremely dry, especially during the night. This is especially true if the eyelid does not completely close. This condition can lead to the swelling of the conjunctiva, which we refer to as chemosis and inflammation of the conjunctiva known as conjunctivitis. Other possible issues that may arise include corneal ulcers and a condition where the cornea gets inflamed, known as keratitis.

Figuring out the root cause and treating it quickly can prevent permanent visual issues such as diplopia, which is seeing double. Some rare complications can include an eye condition known as superior limbic keratoconjunctivitis and damage to the optic nerve, referred to as optic atrophy.

Common problems related to the condition:

Dry cornea due to prolonged exposure
Chemosis – swelling of your eye’s outer layer
Conjunctivitis – inflammation of the outermost layer of the white part of your eye
Corneal ulceration – an open sore on the cornea
Keratitis – inflammation of the cornea
Rare complications such as superior limbic keratoconjunctivitis and optic atrophy

Preventing Exophthalmos

It’s important for patients to know that keeping a regular check on, and taking measures to manage their exophthalmos (a condition where the eyeballs protrude from the eye sockets), can help alleviate symptoms. This attention is necessary alongside the medical or surgical treatment for the root cause of the condition. Treatments like regularly moisturizing the eyes, consistent monitoring, and following the treatment plan set forth by the eye doctor and primary healthcare provider, will ensure that patients receive proper care. All these precautions can contribute to the effective management of this condition.

Frequently asked questions

Exophthalmos, also known as proptosis, is a condition where one or both eyes protrude or bulge out significantly.

The rate of exophthalmos varies depending on the cause.

Some signs and symptoms of Exophthalmos, or bulging eyes, include: - Bulging eyes that can be measured with an exophthalmometer. - Swelling of the eyelid or around the eye, which may be on one or both sides. - Double vision, which can be caused by inflammation or compression of the eye muscles. - Redness in the eyes, which can worsen with bulging eyes and may lead to exposure keratopathy. - Muscle weakness in the eyes, often seen in infectious, inflammatory conditions, or aggressive tumors. - Decreased sharpness of vision. A thorough medical history can help identify the cause of these symptoms. Symptoms such as heat intolerance, weight loss, changes in bowel habits, and palpitations may indicate a thyroid condition. A history of trauma or symptoms like weight loss could point towards cyst growth or a tumor. The speed of symptom onset can also provide clues - quick onset could indicate inflammatory disease, malignant tumors, or carotid-cavernous fistula, while gradual onset could suggest a less severe condition. The presence of pain often points towards an infection. Temporary bulging of the eyes triggered by the Valsalva maneuver could be a sign of blood vessels in the eye being affected. An examination can also help identify the cause of Exophthalmos. This should include checking for diseases such as Graves' disease, leukemia, or tumors in the torso area, as well as any signs that could suggest cancer. A full eye examination is crucial, including checking eye movement, sharpness of vision, pupil response, and eye field. Pressure inside the eye and checks of the front and inner lining of the eye should also be performed. Bulging of the eyes can be quantified using an exophthalmometer, which measures the distance from the front of the eye to the middle of the front edge of the eye socket. It's important for the doctor to be at eye level with the patient during the check. In cases of exophthalmos, the white of the eye below the colored part of the eye is often visible.

Exophthalmos can be caused by various factors, including spreading inflammation into the eye socket, new growths invading the eye socket, disruptions in the flow of blood back from the eye, and foreign objects forced into the eye socket due to trauma or injury.

The other conditions that a doctor needs to rule out when diagnosing Exophthalmos include: - Orbitopathy associated with Graves' disease - Orbital and preseptal cellulitis - Carotid-cavernous fistula - Capillary haemangioma - Neuroblastoma - Leukemia - Lymphoma - Mucocele - Pseudotumors - Secondary spread of cancer from other parts of the body that can affect the orbit - Crouzon syndrome - Apert syndrome - Injuries causing fractures around the eyes leading to bleeding around the eyes and potentially bulging of the eyes.

To properly diagnose Exophthalmos, a doctor may order the following tests: - Blood tests, including a complete blood count (CBC), thyroid function tests, auto-antibody tests, and tests to assess kidney function and inflammation. - Imaging tests, such as a computed tomography (CT) scan, magnetic resonance imaging (MRI), and positron emission tomography (PET) scan. - Biopsy, if necessary, to examine a small sample of tissue from the affected area. - Other tests may be ordered based on the suspected underlying cause of Exophthalmos, such as testing for harmful bacteria in the blood or nose. It is important to note that the specific tests ordered may vary depending on the individual case and the suspected cause of Exophthalmos.

Exophthalmos is treated by addressing the underlying cause of the condition and using various supportive treatments for symptom relief. Close collaboration among eye specialists, primary care providers, and endocrinologists is important, especially when exophthalmos is related to thyroid disease or other secondary causes. Quitting smoking is crucial to prevent and slow the progression of thyroid eye disease, which can lead to exophthalmos. Supportive treatments include using preservative-free eye lubricants, taping eyelids, wearing sunglasses and protective eyewear, and managing double vision with a Fresnel prism or temporary vision blockage. In more severe cases, oral and intravenous corticosteroids may be prescribed to reduce swelling, and surgery may be necessary for cases caused by tissue overgrowth, tumors, or cancerous disease.

The side effects when treating Exophthalmos include: - Dry cornea due to prolonged exposure - Chemosis - swelling of your eye's outer layer - Conjunctivitis - inflammation of the outermost layer of the white part of your eye - Corneal ulceration - an open sore on the cornea - Keratitis - inflammation of the cornea - Rare complications such as superior limbic keratoconjunctivitis and optic atrophy

The prognosis for exophthalmos can vary depending on the underlying cause. In general, any associated swelling, pain, or redness usually improves on its own within 2 to 3 months. However, exophthalmos related to thyroid issues may take longer to resolve and can potentially result in permanent double vision or lasting vision impairment in about 5% of cases.

An ophthalmologist or an eye specialist.

Eye Health Hyperthyroid Eye Disease Intraocular Tumors Ocular Trauma Orbital Cellulitis