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Connecting The Vision - The Ocular Involvement in EDS/HSD

Apr 16, 2023 | Brii Sessions, EDS Moderator | @briisessions | Comments (4)

Author: Mahima Master, MBBS

How can eyes be affected by a connective tissue disorder?

This question needs to be understood and properly evaluated. Collagen works as a building block in connective tissue and occupies almost 80% of our eye structures. Sclera, the protective covering of our eyeball and cornea are mostly made of collagen. EDS, which comprises 13 named subtypes, can be related to genetic mutations in collagen formation.

There are different ocular pathologies associated with EDS based on types of collagens, such as common ocular conditions like myopia, refractive error, glaucoma, keratoconus, dry eyes, and scleral thinning, to more severe conditions, like brittle cornea, scleral rupture, ocular trauma, retinal detachment, and retinal tears. In very rare types of EDS such as vEDS there can be vascular complications due to collagen mutations, which increases the risk of cervical artery dissection leading to blurred vision and visual field defects.

In the hEDS/HSD population, the most common ocular conditions encountered are xeropthalmia (dry eye), and myopia. For patients with hEDS, regular eye checkups help to prevent complications of dry eyes like eye infection, corneal abrasions, and erosions. Due to the collagen alteration in the cornea, EDS patients are prone to a flat cornea that can lead to an increase in axial diameter of the eye causing near-sightedness. Another rare EDS subtype, Brittle Cornea Syndrome (BCS) can increase the risk for corneal thinning due to alterations in corneal curvature leading to strabismus, keratoconus, and risk of ocular injuries.

It is vital to not only to understand the root cause of ocular conditions, but also to screen ocular pathologies in hEDS for effective prevention. At Mayo Clinic, we screen and educate patients regarding various symptoms associated with all types of EDS for comprehensive care. A thorough routine ophthalmologic examination can help understand the symptoms threatening vision. While definitive genetic testing for hEDS/HSD is still a challenge, Mayo Clinic is contributing to the advancement of healthcare by evolving preventive strategies and educating people to understand their condition.

Have you had experience with vision problems? We would love to hear your comments below.

2 Reactions

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passerby | @passerby | Apr 29, 2023

Mine are related to thyroid eye from Graves’ autoimmune. I know the fact that one is still protruding & poor vision after 10 yr has to be due to tissue behind my eye but there’s no fix except invasive surgery.

REPLY
carleenod | @carleenod | Feb 15 9:15am

I am a retired/disabled optometrist. I have just been diagnosed with Lipo-Lymphedema. I will be going for an evaluation for HSD/hEDS soon. I have always had dry eyes, was a -4.50 myope before cataract surgery, and had floppy eyelids. In addition, I always felt I had "soft" corneas because my vision and corneal curvatures varied. I also had phobias that were hard to pin down because of the variabililty. I did not have keratoconus. When I was practicing, I was taught that floppy eye lid syndrome was a sign of possible Obstructive Sleep Apnea. As the years went by I also noticed a change in the appearance of the optic nerves and visual field changes of some of these patients. I'd love to see research done on the ocular signs and symptoms of these connective tissue disorders. As an optometrist I was able to notice things that enabled me to suggest further evaluation with other health care professionals. If more people can be diagnosed earlier, some pain and frustration might be avoided.

REPLY
2 replies
carleenod | @carleenod | Feb 15 9:25am
In reply to @carleenod "I am a retired/disabled optometrist. I have just been diagnosed with Lipo-Lymphedema. I will be going..." + (show)
@carleenod

I am a retired/disabled optometrist. I have just been diagnosed with Lipo-Lymphedema. I will be going for an evaluation for HSD/hEDS soon. I have always had dry eyes, was a -4.50 myope before cataract surgery, and had floppy eyelids. In addition, I always felt I had "soft" corneas because my vision and corneal curvatures varied. I also had phobias that were hard to pin down because of the variabililty. I did not have keratoconus. When I was practicing, I was taught that floppy eye lid syndrome was a sign of possible Obstructive Sleep Apnea. As the years went by I also noticed a change in the appearance of the optic nerves and visual field changes of some of these patients. I'd love to see research done on the ocular signs and symptoms of these connective tissue disorders. As an optometrist I was able to notice things that enabled me to suggest further evaluation with other health care professionals. If more people can be diagnosed earlier, some pain and frustration might be avoided.

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Spell check grr. I meant phorias and double vision. Not phobias.

REPLY
Moderator
Colleen Young, Connect Director | @colleenyoung | Feb 15 8:09pm
In reply to @carleenod "I am a retired/disabled optometrist. I have just been diagnosed with Lipo-Lymphedema. I will be going..." + (show)
@carleenod

I am a retired/disabled optometrist. I have just been diagnosed with Lipo-Lymphedema. I will be going for an evaluation for HSD/hEDS soon. I have always had dry eyes, was a -4.50 myope before cataract surgery, and had floppy eyelids. In addition, I always felt I had "soft" corneas because my vision and corneal curvatures varied. I also had phobias that were hard to pin down because of the variabililty. I did not have keratoconus. When I was practicing, I was taught that floppy eye lid syndrome was a sign of possible Obstructive Sleep Apnea. As the years went by I also noticed a change in the appearance of the optic nerves and visual field changes of some of these patients. I'd love to see research done on the ocular signs and symptoms of these connective tissue disorders. As an optometrist I was able to notice things that enabled me to suggest further evaluation with other health care professionals. If more people can be diagnosed earlier, some pain and frustration might be avoided.

Jump to this post

@carleenod, was it the ocular symptoms that led to your going for an evaluation for HSD/hEDS?

REPLY
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