Graves' Eye Disease

Graves' Eye Disease can be a debilitating condition that in rare instances could result in permanent visual changes. Optimal care is provided by a team of specialist physicians with experience in this relatively uncommon disease. Close coordination among specialists is ideal as patients will often require both medical and surgical treatments over the course of time. Consolidating care at a single institution enhances communication among team members and ensures that care is provided by physicians experienced in Graves' Eye Disease.

Graves' Eye Disease (GED) is an autoimmune condition that causes inflammation of the eye socket, including the fat and muscles which surround the eye. There are many names for this condition, including Thyroid Eye Disease, Thyroid Ophthalmopathy, Thyroid-associated Ophthalmopathy, or Grave’s Orbitopathy.

In this autoimmune disease, the body’s own immune system attacks the tissues which surround the eye, resulting in swelling of the eye. As the surrounding tissues swell, pressure is put on the eye itself, forcing the eyeball to push outwards. This often makes the patient’s eyes more prominent—a condition called “proptosis.”

When the eye itself is pushed outward, the eyelids can have a difficult time closing, resulting in drying out of the eye. Dryness of the eye is irritating and can result in eye redness, excessive tearing, and even scarring of the cornea (eye surface), and permanent visual loss. In very severe cases, swelling of the eye can put pressure on the optic nerve (nerve of vision) resulting in loss of vision—an emergency situation. Additionally, swelling of the eye muscles can occur, which interferes with their ability to move the eye normally—a situation which can result in double vision.

Graves' Eye Disease is characterized by inflammation in the tissues that surround the eye. Common signs/symptoms include:

• Swelling around the eye/eyelids
• Eye pain/headaches
• Bulging or protrusion of the eyes
• Dry eye—because eyelids fail to close
• Redness or irritation of the eye—sometimes with watery eyes
• Decreased or blurry vision
• Double vision

Graves' Eye Disease occurs in patients who have had Graves' disease either currently or in the past. Graves' disease is an autoimmune disease where the body’s immune system attacks the thyroid gland, resulting in a hyperactive thyroid producing excess thyroid hormone. This can cause symptoms such as racing heart, weight loss, sweating, diarrhea, and anxiety. Often patients will present with Graves' Eye Disease within 18 months of diagnosis of Graves' disease, but eye symptoms may precede or lag behind the thyroid disease by years. Upwards of 70 percent of patients with Graves' disease have some degree of eye involvement, but only 3-5 percent will progress to severe disease (roughly 19 per every 100,000 people). Women are much more likely to get Graves' Eye Disease than men.

There are 2 phases of Graves' Eye Disease: An “active inflammatory” phase and a “stable” phase. The active inflammatory phase is characterized by ongoing inflammation of the soft tissues of the eye socket, including muscles, and fat. Patients will experience redness, swelling, and pain typical of ongoing inflammation. Eventually, often with treatment, the active inflammation will subside and patients will enter a stable phase. In the stable phase, the eye structures are no longer inflamed, but remain enlarged, and fibrotic (scarred). The eye will continue to bulge outward and the eyelids may not be able to fully close over the eye. This can result in ongoing dryness/exposure. Enlargement of the muscles may continue as well, resulting in double vision. Lastly, the cosmetic appearance of the eyes may continue to be affected, with a bulging or “staring” appearance.

Cigarette smoking is strongly linked to Graves' Eye disease—such that smokers have 7 times the odds of getting the disease than nonsmokers. Additionally, severe disease is much more likely to occur in smokers than nonsmokers. All patients with Graves' Eye Disease are encouraged to quit smoking immediately.

Medical treatment of Graves' Eye Disease has several goals. In the active inflammatory phase, the goal is to treat the inflammation which is present. This is most commonly done with high doses of oral steroid medications. In some instances, more targeted medications might be useful such as rituximab or other monoclonal antibodies. Medications are usually successful at controlling the inflammation and protecting vision. The second goal, which is present regardless of disease phase, is to protect the function of the eye. This includes preventing the eye from drying out. Excess drying can damage the surface of the eye, resulting in ulcers, and scarring which can permanently affect vision. These treatments could involve lubricating drops, antibiotic drops, or other measures to humidify the eye—particularly while sleeping.

Surgery is rarely indicated during the "active, inflammatory" phase of the disease—where medical treatment is usually sufficient. However, in rare instances, the inflammation is so severe that the optic nerve (nerve of vision) becomes under excess pressure. When this happens, permanent visual loss could occur. In these instances, emergency surgery could be required to remove pressure (decompress) the eye and optic nerve. This is usually done using minimally invasive or endoscopic techniques.

Sinuses after an orbital decompression (removal of the bony walls allows fat from around the eye to settle into the sinuses, relieving pressure)

Most surgery is usually reserved for the “stable” phase of the disease—if the patient continues to have symptoms from protrusion of the eyes, double vision, or unwanted cosmetic changes. The specific surgery is usually tailored to the specific symptoms/problems:

• Orbital Decompression: In this procedure, bone (and sometimes soft tissue) is removed from around the eye. This creates more room, so that the eye can “settle back” into the eye socket—becoming less bulging. This surgery is usually accomplished using small, well hidden incisions or through the nose using an endoscope (no incisions)—with the exact technique determined by our team of oculoplastic and endoscopic surgeons. The procedure can be done either as an outpatient or with an overnight hospital stay. 
• Eye-Muscle Surgery (Strabismus): If the eye muscles have swollen and scarred to different degrees, the patient may be left with ongoing double vision, even after the inflammation has resolved. Following decompression surgery (if necessary), the eye muscles can be adjusted so that they move the eye in a symmetric fashion—improving or eliminating double vision. Because this is a fine-tuning type of procedure, it is usually done in the stable phase after decompression has been done (if necessary) and is usually accomplished in the outpatient setting.
• Eyelid Surgery: Those patients in the stable phase of disease may be left with shortened eyelids. This contributes to the “staring” appearance and makes it hard to close the eyelids and moisturize the eye itself. Outpatient surgery may be required to lengthen or reposition the eyelids—improving function and appearance of the eye.