Coats’ disease, also known as Exudative Retinitis, is an uncommon eye condition which affects the smaller blood vessels (capillaries) found in your retina – the light sensitive layer which lines the inside of your eye. Coats’ disease can make these blood vessels weak and grow incorrectly causing them to leak fluid and blood under your retina. This means that the cells of your retina can’t work properly and this can cause sight to be affected.
Coats’ disease usually occurs in children or teenagers under the age of 18 but most often before the age of 10. It affects boys more than girls, and most children with Coats’ disease only have the retinal changes in one eye.
When you look at something, light is focused onto your retina. The retina is a delicate tissue, made up of light sensitive cells, that lines the inside of your eye. Your retina converts the light into electrical signals that travel along the optic nerve to our brain. The brain interprets these signals so we can “see” the world around us. The retina is supplied with blood by a delicate network of blood vessels on its surface and by a layer of blood vessels underneath the retina.
Light is focused onto a tiny area of your retina called the macula, which is about the size of a pinhead. This highly specialised part of your retina is vital, because it enables you to see fine detail when you are looking directly at something such as words, photos or the television. The macula also gives you much of your ability to see colours. The rest of your retina gives you side vision (peripheral vision).
Coats’ disease affects the smaller blood vessels (capillaries) in your retina. Retinal capillaries are important in supplying your retina with blood which carries nutrients to the cells of your retina so that they work correctly. The cells of your retina need to remain healthy for you to be able to see clearly.
Coats’ disease causes retinal capillaries to develop incorrectly. They become wider (dilated) and twisted, which make them more noticeable when the inside of your eye is examined. The medical term for these changes is telangiectasia.
As well as the retinal capillaries becoming dilated, they also become weak and leaky. This causes some of the fluid from the blood to leak out of your vessels and into your retina. This fluid, known as exudate, builds up in your retina and causes it to become waterlogged (swollen).
Where there are areas of exudates and telangiectasia your retina won’t be able to work properly. This in turn will mean you won’t be able to see clearly in this part of your vision.
Most children with Coats’ disease don’t have any symptoms – the eye doesn’t look unusual, isn’t painful or red, and your child won’t usually be aware that there is a problem. Often children are diagnosed following an unusual finding at an optician’s appointment, after failing a school vision screening test, or when the eye has an odd appearance on photographs like a white/pale pupil rather than a red pupil when a flash photograph is taken.
The cause of Coats’ disease isn't fully known (this is medically known as idiopathic) but it doesn't appear to be hereditary (passed through families) or caused by any other health conditions.
How Coats’ disease affects your child’s vision depends on where in the retina the leaking blood vessels grow. In the early stages of Coats’ disease peripheral (side) vision is more likely to be affected. Peripheral vision describes what you see at the side when you are looking straight ahead.
Coats’ disease may progress and as more of your child's retina is affected, more of their vision can become affected. The macula is the small central area of your retina. It gives you your central and detailed vision, and this is what you use when you look directly at something, for example when you read. If Coats’ disease affects the macula then your child's central vision will be affected.
In the advanced stages of Coats’ disease more vision will have been lost because more of the retina has been affected. When a large area of retina has become swollen it can cause the retina to detach from the back of the eye which can cause loss of sight.
Coats’ disease has different stages depending on how it is affecting the retinal capillaries and retina:
Stage 1: Telangiectasia only, causing little change to the retina and vision.
Stage 2: Telangiectasia and exudation which causes increased changes to the retina and changes in vision.
Stage 3: Large areas of swelling can cause a retinal detachment and this is when the vision is likely to be poor.
Stage 4: The retina can detach completely and there can be other complications which develop, such as secondary glaucoma (raised eye pressure) which can be treated, although sight is still likely to be poor.
Stage 5: Usually at this stage an eye has no sight and no treatment is available to improve sight. In most cases the eye isn’t painful, but if it were, treatment may be needed to prevent the pain or further damage to the eye.
An eye examination is the only way to tell if your child has an eye condition. Children can adapt very quickly to a change in their vision and there can be very few signs that they have any problems especially if only one eye is affected. All children should have their eyes screened when they start full time at school.
If you are concerned about your child's vision or their eyes it’s important to have them checked by an optometrist (optician). They are able to check any changes in the level of your child's vision and the health of their eyes. If the optometrist is concerned by anything they find during the eye examination, they will refer your child to the eye clinic, at the hospital.
At the eye clinic, your child's eyes will be checked by an eye specialist, known as an ophthalmologist. The ophthalmologist will put drops into your child's eyes which will make their pupils bigger to allow them to get a better view when examining the retina. They will then shine a bright light into your child's eye to look at the retina. It’s normal for your child to feel that the light is too bright and that it’s uncomfortable, but it’s reassuring to know that it won’t cause any damage to your child’s eye.
Sometimes the ophthalmologist may want to perform another test known as a Fluorescein Angiogram. This is where a dye is injected into a blood vessel in your child's arm, which travels through their blood stream to their eye. When the dye reaches their eye a series of photographs will be taken which will show the blood vessels of their retina filling with the dye. From these photographs, the ophthalmologist will be able to see if any of these capillaries are leaking and be able to let you know if your child has Coats’ disease.
The changes that occur to the retina in Coats’ disease can be treated to try and prevent further changes to your child’s sight.
In the early stages of Coats’ disease there may be little or no effect to your child’s vision. If this is the case the ophthalmologist may decide to monitor your child’s eyes at regular appointments rather than do any treatment. In some children Coats’ disease doesn't develop any further than stage 1.
In later stages, when the Coats’ disease has more of an affect on your child’s retina, the ophthalmologist may decide to do some treatment to help prevent further changes to your child’s sight.
The treatment which your child may have depends on where and how much of their retina has been affected. The treatments used for Coats’ disease are laser photocoagulation and cryotherapy (freezing treatment). The aim of both these treatments is to stop the retinal capillaries from leaking. Most children would have these treatments under a general anaesthetic.
In more advanced stages of Coats’ disease, where the retinal capillaries have leaked a lot and the large areas of swelling have caused the retina to detach from the back of the eye, the treatment will be aimed at reattaching the retina. The type of treatment may vary depending upon how large the detachment is and how long the retina has been detached for.
The recent research for the treatment of Coats’ disease has looked at the possible use of anti-VEGF drugs, which can be used to prevent the development of new leaking blood vessels. These treatments are used regularly in adults for other eye conditions but their use is Coats’ disease is relatively new. Coats’ disease usually has to be treated on a very individualised basis and your ophthalmologist will be able to advise whether or not anti VEGF treatment would be an option for your child. At the moment this treatment is not available on the NHS, though your ophthalmologist may be able to arrange funding. A lot more research may need to be done before they can be used routinely for everyone with Coats’ disease.
The effect of Coats’ disease on sight will vary from person to person. Generally the condition will gradually progress and affect more of the retina. For younger children, especially those under 3 years old, the changes to their retina tend to progress more quickly, are difficult to control and are more likely to have a long term effect on visual development. In older children and young adults, Coats’ disease can have a much milder and slower effect on the retina. In many cases this progression may stop on its own and may not need any treatment.
The effect that Coats’ disease will have on your child’s sight will depend on the age your child is when it’s first diagnosed. Being diagnosed early when the capillaries are only showing slight changes would mean that there are more treatments available to your child, like laser photocoagulation or cryotherapy. In some cases treatment can improve vision.
If your child has been diagnosed in the later stages of Coats’ disease, where large areas of the retina have already been affected, then laser photocoagulation or cryotherapy treatment may not be suitable.
If your child has had a retinal detachment, then there are different treatments available to help repair this. It is important that a retinal detachment is repaired quickly, as this can help with how successful the treatment is.
Sometimes despite all treatment, the eye doesn’t respond and may lose all sight. Late complications such as cataract (cloudy lens) or glaucoma (raised pressure) may need treatment. Typically the eye stays comfortable but very rarely, if the eye remains painful it could be removed but this would be a last resort.
Most children with Coats’ disease do not have their eye removed.
The amount of vision lost will vary between individuals. Coats’ disease usually affects one eye only (monocular vision) and children tend to adapt very well to using their better eye. This doesn’t mean that they will be over using their better eye or causing any damage to their better eye. It’s unusual for children with good vision in one eye to need additional support in their education and they are not considered partially sighted.
Having monocular vision can affect your child's depth perception and hand-eye coordination. For some tasks your child may appear clumsy and uncoordinated at first, for example when they throw and catch a ball. It’s reassuring to know that this generally improves when your child gets older and adapts to being monocular.
Even with good vision in their better eye, your child doesn't have as much peripheral vision as someone with two eyes. With time they will adapt to this without realising by turning their head to see things in their peripheral vision. You can help your child by sitting or approaching your child on the same side as their good eye. This would make it easier for your child to see you.
These are difficulties your child may experience and you might find it useful to inform their nursery, school teachers or carers of these.
In the future your child would still be able to drive a private vehicle, as long as the vision in their remaining eye is unaffected by other eye conditions and they are able to achieve the visual requirements for driving. However monocular vision would mean your child couldn't hold a Heavy Good Vehicle or Public Service Vehicle licence. Some professions, such as being a pilot, policeman or some areas of the forces require a certain level of vision to be reached in both eyes. Keeping this in mind can help you and your child plan their career choice for the future.
It’s completely natural to be upset when your child has been diagnosed with Coats’ disease and it’s normal to find yourself worrying about the future and how your child will manage with a change in their vision.
It can sometimes be helpful to talk over some of these feelings with someone outside your circle of friends or family. At RNIB, we can help with our telephone Helpline and our Emotional Support Service. You may also find your GP or social worker can help you find a counsellor if you feel this might help you.
Whether your child has just been diagnosed with Coats’ disease or has been living with it for a while, at RNIB, we are here to help and support you through your journey.
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Look UK is an organisation which helps support families with children aged between 0-16 with vision problems. They have family support officers and help support families at home and in school.
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