If you or your child has been diagnosed with Ehlers-Danlos syndrome, there's no doubt you have a list of medical specialists to see, such as dermatologists, rheumatologists, and physical therapists. Additionally, you'll need to also see an optometrist or an ophthalmologist for a comprehensive eye exam. Why? Because Ehlers-Danlos syndrome (EDS) is a connective tissue disorder due to not having enough collagen, which is the glue that holds everything together in the body. The eye is 80% collagen. Here's what you need to know.
Know the eye conditions and symptoms related to EDS
Due to the lack of collagen in your eyes, your eyes and vision can be affected. EDS can cause the whites of your eyes to turn blue. Your vision may be blurry, or one eye may drift or cross. You may see floaters in your field of vision, and your eyes may be sensitive to light. Due to the hyperelasticity of your skin, your eyelids may have folds of skin.
While those symptoms can be irritating, some are treatable, such as by wearing prescription lenses with prisms to correct your vision. However, the following symptoms may be life-changing.
- Glaucoma. Pressure inside the eye can increase due to lack of collagen. This causes changes in the anatomy of the eye and can lead to glaucoma, which is irreversible and may require surgery.
- Detached retina. The retina is what directs rays of light to be focused in your eye. The retina can detach due to the lack of collagen in the eye, causing the eye to expand, lengthen, or change shape. This may be corrected with surgery.
- Macular degeneration. The macula is the part of your eye that has the highest concentration of receptors. Macular degeneration occurs when the blood vessels break or leak and cause the macula to atrophy. If not corrected, such as with medication or therapy, this condition can cause blindness.
Establish a baseline with a comprehensive eye exam
It's important to know that EDS is progressive. Symptoms of the condition will worsen throughout your body, and that includes your eye symptoms. Because of this, it is crucial that you establish a baseline with every medical specialist you will see as soon as possible after getting diagnosed with EDS. In regard to your eye care, a baseline can be established through an eye exam and used to determine whether any significant changes occur in between subsequent visits.
Any changes that are noted will be marked as an additional baseline moving forward, especially when it comes to irreversible damage to your eyes. This can help your eye doctor determine how progressive your condition is so he or she can treat you appropriately.
Watch for comorbid conditions that can also affect eyesight
There are a number of comorbid medical conditions that can go along with EDS in some way. Chiari malformation is one of them. It's believed, though not proven, that some people with EDS have Chiari malformation due to the lack of collagen in the body causing the lower part of the brain to be lower than it should in the skull.
Chiari malformation also affects the eyes with blurred vision, double vision, or nystagmus (involuntary eye movement). It's important to get tested for Chiari if you have these eye symptoms or pressure headaches in the back of your head. Testing for Chiari malformation is done with an MRI of the head and neck. If diagnosed, you'll need to see a neurologist or a neurosurgeon.
Be sure to inform each of your medical specialists that you have EDS, particularly before you have surgery anywhere in your body. The inability for your body to produce enough collagen may make it more difficult for your body to heal from having surgery.