Orbital Pseudotumor - Causes, Symptoms, Diagnosis, and Treatment

Introduction:

Have one ever seen anyone experiencing a painful mass in their eye region? Wondered what it could be. Fortunately, not all painful eye masses are tumors. Some are benign growth masses that could have no systemic or local causes. These benign, painful growths in the orbital region are called orbital pseudotumors. The word ‘pseudo’ simply means 'false.' As the name suggests, orbital pseudotumors mimic tumors but are not cancerous. They do not usually diffuse to other regions of the body as well.

What Is an Orbital Pseudotumor?

Orbital pseudotumor is a benign, non-infectious, non-neoplastic, space-occupying condition characterized by a painful mass growth in the orbital region. The orbit is the hollow portion in the frontal part of the skull that contains eyeballs and tissues that support the eyes' function. There is no specific known local or systemic cause for orbital pseudotumor yet. Therefore, orbital pseudotumor is also known by other names, such as idiopathic orbital inflammatory syndrome (IOIS), nonspecific orbital inflammation, or inflammatory orbital syndrome.

What Are the Causes of Orbital Pseudotumor?

Orbital pseudotumor is commonly called an idiopathic orbital inflammatory syndrome (IOIS). As the name suggests, there is no known clear cause for orbital pseudotumor yet. About 8 to 11 percent of orbital inflammations are reported to be orbital pseudotumors. No local or systemic causes are known to cause orbital pseudotumor. However, certain infections like streptococcal infections, upper respiratory tract viral infections, rheumatoid arthritis, ulcerative colitis, and Crohn's disease are suspected to be associated with orbital inflammatory diseases.

Who Can Be Affected by Orbital Pseudotumor?

Studies show that people between the ages of 30 and 60 are known to be affected by orbital pseudotumor more commonly. Females are more likely to be affected by orbital pseudotumor than males. Orbital pseudotumor is very rare in children.

What Are the Common Signs and Symptoms of Orbital Pseudotumor?

Idiopathic orbital inflammatory syndrome or orbital pseudotumor is a painful tumor-like growth.

• Painful swelling in the orbital region in and around the eyes.

• Initial itching of the eyes has been reported in some cases.

• Slow-growing painful mass in the orbital region.

• Exophthalmos is a common finding. The eyes protrude out of the orbit as the tissues in orbit are swollen.

• Blurred vision.

• Double vision (diplopia).

• Sensitivity to light.

• Restricted eye movements.

• Headaches that are recurrent or persistent.

• Symptoms are usually unilateral. However, bilateral orbital pseudotumors are seen in children.

How Can Orbital Pseudotumor Be Diagnosed?

Clinically, the signs and symptoms of orbital pseudotumor resemble orbital tumors. However, the presence of orbital tumors has to be ruled out to confirm the diagnosis of orbital pseudotumor.

• Blood tests like complete blood cell count (CBC), ESR (erythrocyte sedimentation rate, and C-reactive protein tests.

• Magnetic resonance imaging (MRI) is done.

• Computed tomography (CT) scans are performed.

• Ultrasound imaging can be done to view the extent of the orbital pseudotumors.

The treatments are started right after the accurate diagnosis of orbital pseudotumor is made.

What Other Conditions Resemble Orbital Pseudotumor?

Many other conditions resemble orbital pseudotumor. Some of the conditions that resemble orbital pseudotumor are as follows:

1. Orbital infections may lead to orbital cellulitis.

2. Thyroid ophthalmopathy is a systemic condition that mimics orbital pseudotumor.

3. Certain severe malignant conditions like leukemia, lymphoma, and metastatic carcinoma resemble orbital pseudotumor.

4. Sarcoidosis.

5. Rheumatoid arthritis and other autoimmune disorders like systemic lupus erythematosus might present with the signs and symptoms of orbital pseudotumor.

6. IgG4-related orbital diseases might mimic orbital pseudotumor.

The conditions mentioned above are some of the conditions that mimic orbital pseudotumor. Therefore, severe conditions like lymphoma and other malignancies should be ruled out before starting treatment for orbital pseudotumor.

What Are the Treatment Methods for Orbital Pseudotumor?

Orbital pseudotumors are often slow-growing, painful tumors. They do not usually metastasize to other parts of the body. Though they resemble orbital tumors, orbital pseudotumors are often fully curable. A decent amount of persons affected by idiopathic orbital inflammatory syndrome (IOIS) are known to be cured without any treatment. Steroids are known to show dramatic improvements in the cure of orbital pseudotumor. The pain is known to subside within 48 hours after administering corticosteroids.

Some of the standard treatment methods are discussed below,

• The use of corticosteroids helps manage the pain and prevents the growth of orbital pseudotumor.

• Radiation therapy.

• Nonspecific steroid-sparing agents like Methotrexate, Cyclosporin, Cyclophosphamide, and Rituximab are used to treat orbital pseudotumors.

• Specific biologic agents are also widely used in treating orbital pseudotumors. Biologic agents like Infliximab, Adalimumab, and Abatacept are used in treating orbital pseudotumors.

• The most widely used steroid in managing orbital pseudotumor is Prednisone, administered 1 mg per 2.20 pounds of body weight once a day for adults.

• Though orbital pseudotumors are benign, they are known to cause severe effects like visual impairments or even loss of vision if not treated properly. In severe cases, steroids are administered intravenously until improvement is seen.

These are some of the standard methods used extensively in treating orbital pseudotumor. Over 75 percent of the people affected by orbital pseudotumor are reported to show improvements within 24 hours to 48 hours of the treatment. Also, more than 50 percent of patients are known to have recurrent orbital pseudotumors after treatment.

What Are the Complications of Orbital Pseudotumors?

Orbital pseudotumors can exert significant pressure on the eyes, potentially damaging the surrounding tissues. This pressure can push the eyeball forward so that the eyelids can no longer cover or protect the cornea, the eye’s outer clear layer. Without this protection, the eye may become very dry, which can result in cloudiness and even corneal ulcers. The pressure from orbital pseudotumors can also impact the eye muscles, limiting their movement and disrupting the coordination of eye movements. This can cause double vision and restricted eye movement. Without proper treatment, or if the condition does not respond to therapy, an orbital pseudotumor can lead to vision loss. Inflammation from the pseudotumor can spread to nearby structures, and severe cases may result in secondary angle-closure glaucoma.

When Should Individuals Contact a Medical Professional?

Individuals with this condition require regular follow-up care with an eye doctor experienced in treating orbital diseases. Contact the healthcare provider immediately if the person experiences any of the following issues:

• Decreased vision.

• Corneal irritation.

• Pain.

• Redness.

Conclusion:

Orbital pseudotumors are benign growths in the orbital area. However, in some cases, if left untreated or in some cases when the orbital pseudotumor is unresponsive to the therapy, it may lead to severe effects like vision loss, or the growth may become contagious and might affect the adjacent structures and nerves. Therefore, proper treatment and diagnosis are required to avoid the complications of orbital pseudotumor.