Also known as Graves’ Orbitopathy, this is an inflammatory disease of the orbital tissues. It is often but associated with an overactive thyroid gland.
What is thyroid eye disease?
It is an autoimmune disease. This means that the body’s immune system is inappropriately producing antibodies against thyroid gland tissue and orbital muscle and fat. In other words the immune system is attacking the body’s own tissue. It is not known why this happens. There are many different autoimmune diseases that affect almost all the different tissues in the body. Rheumatoid arthritis is another such condition, where the immune system attacks the cartilage in joints causing joint inflammation: swelling and pain. Autoimmune diseases follow similar courses. They have an active phase when inflammation is present followed by an inactive phase when there is no inflammation. Active inflammation usually means the following symptoms and signs: pain, swelling, redness, loss or altered function of the affected tissue e.g. double vision when eye muscles are inflamed. Inactive inflammation may mean a complete recovery to normal, however in some people the active inflammation causes scarring or destruction of tissues. When the inflammation becomes inactive there may be permanent loss of function or damage caused by this scarring e.g. persistent double vision due to scar tissue in eye muscles in Grave’s orbitopathy or a joint that won’t bend properly in rheumatoid arthritis. Usually symptoms of an overactive thyroid gland precede the orbital problems but not always. For some, the first sign of any problem is orbitopathy (eye problems). Your care is usually coordinated between an endocrinologist, an orbital surgeon and sometimes an endocrine surgeon. You may also be treated with Radio Iodine in a Nuclear Medicine Department.
What are the symptoms and signs of thyroid eye disease?
Your eyes may feel swollen, sore, watery, and gritty. Your vision may become blurred. The ability to see colour may be different in one eye compared to the other. A reduction in vision or a difference in colour perception between the two eyes may be a sign of optic nerve compression. This is an emergency and you should see an ophthalmologist urgently. The eyelids may become swollen and red. The white of the eye may become red. You may have pain in and around the eye. You may experience pain on eye movements. The muscles that move the eye become swollen and may limit how well the eye moves. This in turn may cause double vision (seeing two images). If you have double vision you should not drive. Contact the DVLA for advice. The eyeball may protrude or bulge forwards. The eyelids may retract. The upper lid is pulled upwards and the lower pulled downwards. This causes the white of the eye to be revealed above and below the coloured part of the eye. This may produce a staring eye appearance.
How is smoking linked to thyroid eye disease?
If you smoke, the condition is likely to be more severe. If you continue to smoke you are more likely to have a flare up or return of active inflammation. If you continue to smoke any flare-ups you have are likely to be more severe. The overwhelming evidence is that you should stop smoking if you have this disease. It is difficult to stop smoking however there is hard evidence that smoking makes this condition worse and every effort must be made to stop. Most people are not successful when trying to stop on their own. Most success stories are those who seek help through a smoking cessation programme from their GP or using nicotine replacements. Click here for help on smoking cessation.
What tests may be carried out to determine if I have thyroid eye disease?
Mr McCormick will examine you in clinic, making measurements of your eyes and vision. You will also see an orthoptist. This is a health care professional that specialises in eye movement disorders. They will take very careful measurements of your eye movements and note any double vision that you have. The may also be able to help with double vision, either by applying a stick on prism to spectacles or with occlusion of one eye. You will need to have a blood test. The thyroid hormone levels and antibodies to thyroid gland tissue will be assessed. If at any point you require treatment with immunosuppressant drugs (steroids, Azathioprine etc.) you may need to have more blood tests before and during treatment. In addition, blood pressure and the skin prick test for diabetes may be used before and during steroid therapy. A CT or MR scan of the orbits is usually carried out.
How is thyroid eye disease treated?
Firstly the thyroid gland hormone levels must be brought under control. An endocrinologist does this. Usually this occurs before orbitopathy problems but they may occur at the same time or visa versa. Control of thyroid hormones may be with drugs, radioactive iodine drink or thyroid gland surgery. For orbitopathy there are two stages of treatment depending on disease ‘activity’.
If inflammation is active, the treatment is aimed at suppressing this inflammation but only if severe enough. Most people have mild disease that doesn’t require immunosuppression. Types of immunosuppression include: oral steroids, intravenous steroids, Azathioprine and orbital radiotherapy. Oral steroids start at a high dose and then gradually reduce over a period of months. Intravenous steroids are given via a drip. They may be given as a day case and repeated 1 week later. This may continue weekly, sometimes for up to 12 weeks. It is discontinued if no response to treatment is seen. Azathioprine is started at a low dose and gradually increased according to side effects and response to treatment. Localised orbital radiotherapy is delivered in small doses by an oncologist. It acts by suppressing the inflammation in the orbit by inhibiting the cells that cause inflammation. Although radiotherapy is usually used for cancer treatment it is an effective anti-inflammatory treatment. The time to maximum effect for the treatment is 6 weeks so you may need to be on steroids as well during the intervening period. The side effects, risks and benefits of each of these treatments will be discussed with you in clinic. Rarely, if vision is threatened by compression of the optic nerve in active inflammation, an orbital decompression may be required. Click here for details of this operation. If you have double vision this may be treated with a prism in spectacles or patching one eye. This is performed by an orthoptist in clinic. The prism used first of all is called a Fresnel prism and is stuck onto your existing glasses (or a new pair if you don’t currently wear them). Eventually, if the double vision is stable, the prism may be incorporated into the glass lens in spectacles.
The second stage is inactive inflammation. After the inflammation has become inactive you will be reassessed to determine if surgery to rehabilitate you may be of benefit. This is discussed below.
What operations are performed for thyroid eye disease?
As already mentioned, rarely an orbital decompression is required in the active inflammation stage, when sight is threatened and immunosuppressants have failed to control the disease. Most surgery however is carried out in the inactive inflammatory phase. The surgery appropriate for you will depend on what problems you are having: most do not require any surgery. An orbital decompression is carried out to reduce the amount of proptosis or protrusion of the eyeball. Often eyelid retraction improves as well but not always. Symptoms of pressure behind the eye and pain on eye movements may also improve, in addition to eyelid closure and therefore improve ocular surface symptoms such as grittiness and dryness. Click here for details of orbital decompression surgery. Strabismus or squint surgery may be performed if you have double vision. A colleague of Mr McCormick’s, Mr Ian Marsh is the regional expert in double vision surgery. He also works at Aintree NHS Trust and Spire Liverpool.
Eyelid retraction surgery aims to correct the retracted eyelid position by raising a lower eyelid or dropping an upper lid. Click here for details of eyelid retraction surgery.
Blepharoplasty surgery is performed to reduce the protrusion of fat from the orbit into the eyelids and to remove any excess skin. Click here for details of blepharoplasty surgery.
The order in which surgery is carried out is as follows: orbital decompression; strabismus (squint) surgery; eyelid surgery, although not everyone requires all of these treatments. Usually one side of surgery is carried out at a time. After orbital decompression surgery a period of at least 4-6 months is required before strabismus surgery may be performed. This is to allow the inflammation and swelling caused by orbital surgery to settle. This means that if you go ahead with all of the surgery available for Grave’s orbitopathy it may take several years. Most people however do not need all and treatment is tailored to their individual problems.
What is orbital decompression?
The orbit is the bony eye socket that contains the eyeball, eye muscles and lacrimal gland. There are also nerves, arteries, veins and orbital fat. People who have thyroid eye disease may experience a problem called proptosis. This is when the eyeball protrudes forwards out of the eye socket. Orbital decompression is an operation to remove fat from around the eye and to increase the space inside the orbit to allow the eyeball to move back into place. The space is increased by drilling away bone from the walls of the orbit and creating windows in the bony walls, into the air filled nasal sinuses so that orbital tissues may fill this extra space. It is usually carried out routinely to improve symptoms of pressure behind the eye, exposure of the surface of the eye and the unsightly staring appearance. It is rarely carried out as an emergency to save eyesight if the inflammatory phase of thyroid eye disease has not responded to steroid treatment and the nerve of vision is threatened.
Top - a photograph of a skull with an arrow pointing to the medial wall of the orbit and the ethmoid sinuses, which are removed in a medial wall decompression; Bottom - a photograph of a skull with an arrow pointing to the lateral wall of the orbit, which is partially removed in a lateral wall decompression
What symptoms and signs may improve with orbital decompression surgery?
Symptoms of pressure behind the eye, discomfort on eye movement and dry eye should improve with this operation. The protrusion (proptosis) of the eye should improve as it moves back into the eye socket. Initially this may not be noticeable due to swelling in the orbit caused by the operation itself. After 3 months there should be a noticeable improvement but the eye can continue to move back for up to a year after surgery. When the eye moves back the eyelids may also move back into position and reduce the amount of white of the eye that is visible. This is not always completely resolved and some people require eyelid retraction surgery at a later date. Double vision is not usually improved with this operation but in some cases it may be. The operation itself carries a small risk of creating or worsening double vision. For this reason it is good practice to carry out orbital decompression surgery before any double vision surgery. The eyeball will be less exposed. In other words the ability of the eyelids to move over the eye, close and distribute tears should improve. This improves the symptoms of dry eye: burning, grittiness, dryness, and photophobia.
What are the risks and possible complications of surgery?
All surgery caries a risk of bleeding and infection, both are fortunately uncommon in this operation. Infection might present as increased swelling and redness of the skin. There might also be yellow discharge from the wound. It is treated with antibiotics. Bleeding may present as fresh blood oozing from the site of surgery or a lump appearing near the wound after the operation. Simple pressure on the area is usually enough to control minor bleeding. A haematoma collecting in the orbit, behind the eye, may compress the nerve of vision and threaten eyesight. It is extremely rare for this to occur. It presents as pain, loss of vision and a bulging forwards of the eyeball. It is an emergency and as such is initially treated in an accident and emergency department where Mr McCormick or an on call ophthalmologist will attend to you. Any orbital surgery carries a very small risk of permanent loss of vision. This could be due to damage to the nerve of vision during the procedure or bleeding after the operation. Any orbital surgery carries with it a risk of double vision. In most cases, if this occurs, it is temporary but rarely it is permanent. Permanent double vision may be corrected with surgery or prisms in spectacles. Sensory loss (numbness) may be a temporary problem after surgery affecting the cheek, side of nose, upper lip and upper teeth. This is due to swelling of a nerve that travels in the bone of the floor of the orbit close to where bone is removed. In rare cases the nerve is permanently damaged and if so the numbness will not improve. Whenever the skin is incised a scar may form. Every attempt is made by Mr. McCormick to minimise and hide scars but sometimes they can be visible. You will have the opportunity to discuss the risks and benefits of surgery and anaesthesia with Mr McCormick and the anaesthetist prior to surgery.
What will happen before surgery?
Before the operation Mr McCormick will see you in the clinic. He will ask you about the orbital problem. He will also ask about other medical problems you have, medications you take and any allergies (bring a list or the tablets themselves with you). He will examine your eyes. If you are to proceed with surgery the operation will be discussed in detail. This will include any risks or possible complications of the operation and the method of anaesthesia. You will be asked to read and sign a consent form after having the opportunity to ask any questions. You will need to have a CT scan of the orbits. Mr. McCormick will book this if it has not already been carried out as part of your trauma assessment. You will also see a preoperative assessment nurse. She/he will carry out blood tests and an ECG (heart tracing) if required. She/he will also advise when you need to starve before the operation.
What should I do about my medication?
Mr. McCormick will want to know all the medication that you take and about any allergies you have. In some cases you may be asked to stop or reduce the dose of blood thinning tablets like: warfarin, aspirin, clopidogrel (plavix), dipyridamole (persantin), apixaban, dabigatran. This decision is made on an individual basis and you should only do so if it is safe and your GP, surgeon or anaesthetist has instructed you. This will be discussed with you before surgery. You should avoid non-steroidal anti-inflammatory medications for 2 weeks prior to surgery. Other medication should be taken as usual. You should avoid herbal remedies for 2 weeks prior to surgery as some of these may cause increased bleeding at the time of surgery.
What should I expect after surgery?
Usually a pad will be placed on the eye, which will remain until the following day when you can remove it. In some cases your eye will be examined at regular intervals after surgery to make sure there are no signs of post-operative bleeding. For 10 days the wound should be cleaned using boiled water that has cooled down and sterile cotton wool balls. After the pad is removed, antibiotic ointment should be applied to the skin wound and the eye three times a day for 2 weeks. It is normal for there to be swelling of the eyelid following surgery. This gets worse over the first 48 hours then starts to improve. It may take up to 6 weeks to resolve. Usually the skin sutures used are dissolvable and will be left to fall out by themselves. The time when you can return to normal activities varies from person to person. For some it is 1 week, others 2 weeks.
If an eye pad is placed it should remain until the next morning when you may remove it. For the first 10 days clean the wound using either sterile water sachets or boiled water that has cooled down and sterile cotton wool balls. Chloramphenicol ointment to the eye and wound, three times a day for 2 weeks. You can also use this to massage the lower eyelid in an upward direction. This is to counter lower lid retraction and should begin at day 7. Oral antibiotic for 5 days. Oral steroid (prednisolone) 60mg 2 days, 40mg 2 days, 20mg 2 days, 10mg 2 days then stop. No nose blowing for 6 weeks. No hot drinks for 48 hours. Sleep propped up at 45 degrees for 48 hours. Follow up appointment 1 week later.