What is a detached retina?
Retinal detachment is a term used to describe when the thin layer of tissue (the retina) pulls away from the back of the eye. As seen in the photo below, seeing tiny specks that float through your field of vision (floaters), blurred vision, and flashes of light in one or both eyes are common warning signs of this condition.
Why does it happen?
People with Smith-Magenis Syndrome are at an increased risk of retinal detachment. This is due to the high frequency of myopia, combined with self-injurious head-banging, and hyperactivity.
A case report “Eye abnormalities in the Smith-Magenis contiguous gene deletion syndrome” was published by BM Finucane, E R Jaeger, M B Kurtz, M Weinstein, and C I Scott Jr, in 1993 on the PubMed.gov website explaining this a bit further. An abstract from this article states:
We present the results of ophthalmologic assessment in 10 patients with interstitial chromosome deletions of 17p11.2, otherwise known as the Smith-Magenis syndrome (SMS)… …Ocular pathology appears to be very common in SMS, significantly contributing to disability in people with this syndrome. The combination of high myopia, self-injurious head-banging, aggression, and hyperactivity among these patients makes them particularly susceptible to retinal detachments.
Eye abnormalities in the Smith-Magenis contiguous gene deletion syndrome
The link between myopia and retinal detachment is explained further by Katie Williams and Christopher Hammond in their article “High myopia and its risks” published in the Community Eye Health Journal in 2019:
The risk of developing a retinal detachment is five or six times greater in people with high myopia (OR >20) compared to those with low myopia (OR <4). People with high myopia have longer eyes (axial elongation), which means that the retina is more stretched and therefore prone to peripheral retinal tears. In addition, myopic eyes have a degenerate vitreous that is more likely to collapse and separate from the retina, also increasing the risk of retinal tears. High myopia can also cause central retinal degenerative changes such as posterior staphyloma, lacquer cracks and chorioretinal atrophy; these have been used to grade myopic maculopathy.
High myopia and its risks, Katie Williams and Christopher Hammond
What are the symptoms?
Often if an individual with Smith-Magenis Syndrome is experiencing a detached retina, they will:
- Become very light intolerant.
- Avoid looking at sudden or persistent flashing lights.
- Unable to easily walk around due to their blurry or reduced vision.
- Individuals may rub their eyes more in an attempt to clear their vision.
Strategies to prevent retinal detachment
The potential for retinal detachment or damage is increased in SMS due to self-injurious behaviours. Therefore strategies to minimise the behaviour (where possible), or damage to the retina, should be adopted. Some strategies to consider could be:
- Talk to a pyschologist who may be able to help and advise with behaviour strategies to prevent self-harm
- Soft helmets are widely available and can be used to protect the head from self-injurious behaviours. Talk to your GP or paediatrician to see what is available through the NHS.
It is important that if you suspect retinal detachment, you seek immediate medical attention.
Treatments for retinal tears and detachments
A retinal tear is a tear or hole that hasn’t yet progressed to detachment, an eye surgeon may suggest one of the following procedures to prevent retinal detachment and preserve vision:
- Laser surgery (photocoagulation). The surgeon directs a laser beam into the eye through the pupil. The laser burns around the retinal tear, creating scarring that usually “welds” the retina to underlying tissue.
- Freezing (cryopexy). After administering a local anesthetic to numb the eye, the surgeon applies a freezing probe to the outer surface of the eye directly over the tear. The freezing causes a scar that helps secure the retina to the eye wall.
Both of these procedures can be done on an outpatient basis. After the procedure, the advice will usually be to avoid activities that might jar the eyes — such as running — for a couple of weeks or so.
If the retina has completely detached, surgery will be needed to repair it, preferably within days of a diagnosis. The type of surgery the surgeon recommends will depend on several factors, including how severe the detachment is:
- Injecting air or gas into the eye: In this procedure (pneumatic retinopexy) the surgeon injects a bubble of air or gas into the center part of the eye (the vitreous cavity). This bubble pushes the area of the retina containing the hole or holes against the wall of the eye, stopping the flow of fluid into the space behind the retina. Fluid that had collected under the retina is absorbed by itself, and the retina can then adhere to the wall of the eye. The patient may need to hold their head in a certain position for up to several days to keep the bubble in the proper position, and the bubble eventually will reabsorb on its own.
- Indenting the surface of your eye. This procedure, called scleral buckling, involves the surgeon sewing (suturing) a piece of silicone material to the white of your eye (sclera) over the affected area. This procedure indents the wall of the eye and relieves some of the force caused by the vitreous tugging on the retina.
If there are several tears or holes or an extensive detachment, the surgeon may create a scleral buckle that encircles the entire eye like a belt. The buckle is placed in a way that doesn’t block vision, and it usually remains in place permanently.
- Draining and replacing the fluid in the eye: In this procedure (vitrectomy) the surgeon removes the vitreous along with any tissue that is tugging on the retina. Air, gas or silicone oil is then injected into the vitreous space to help flatten the retina. Eventually the air, gas or liquid will be absorbed, and the vitreous space will refill with body fluid. If silicone oil was used, it may be surgically removed months later.
References
About the Author
Liam went from being able to read and write in perfect 20/20 vision, completing his GCSE examinations in 2017, to having someone else read his results a mere few months later, on results day. He suffers from Leber’s hereditary optic neuropathy (LHON), which is the acute or subacute loss of central vision predominantly affecting young adult males. There is no pain.
Liam’s Story…
I am 19 years old and still adapting to my central vision loss, writing this blog is a reminder of my adaptations over abnormality. As you read this, attempt to do so with both thumbs up in front of the centre of your eyes…. difficult right? This is reminiscent of my experience of having central vision loss.
Despite having the experience of dealing with sight loss it has taught me the fundamental coping method of having a disability. It’s the ability to adapt. Over time, and with persistence, that experience simply becomes normal. As I write this my screen is zoomed in four times. I have learned to touch type and use technology such as ‘text to speech’ that enables me to write this blog . As quickly as I lost my vision, I found I had to quickly to adapt to these changes.
With all forms of life-altering events, it does present some form of psychological torment. But in my experience, my mother felt the hardship of loss more than I did. I was caught up in adapting to my disability and spared little time thinking about the emotional consequences of the situation.
My family had a sense of hurt due to not being able to have control of the situation. I suppose nothing is harder than always being the figure who can solve problems for your child, to not being able to solve this problem. Fully understanding the problem can help with solving the emotional aspects of a disability. My adaptations ‘normalised’ my newfound disability. A combination of professional advice, along with my experience and adaptations, helped my family gain understanding and a real sense of reassurance that everything was going to be okay.
Cortical Visual Impairment