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Subluxed lens & 2nd RD risk - ? Marfan's, Ehlers Danlos folks...

Hello everyone! I am an adult woman with Ehlers Danlos (a connective tissue disorder). I have eye issues (understatement) and a question please for those with experience - either fellow patients or doctors. I am very myopic. Sixteen years ago I had a retinal detachment in my left eye, which was successfully reattached  with laser surgery (vitrectomy and gas bubble). I am aware that eye surgery often causes other problems later. Two years ago the lens in the left eye subluxed (ectopia lentis). I was left with higher-order visual problems in the eye (double vision, blurriness, glare etc.) but have made do with a very high prescription contact lens. My other eye is corrected to 20/20+ and healthy, for which I am deeply grateful. A few weeks ago I accidentally bumped my head, and the lens shifted again. I am considerably more impaired and am discussing a lensectomy with my doctor. He does not have experience with connective tissue disorder. I am trying to assess my level of risk in opting for this operation, as I know I could easily have a second retinal detachment, and possibly lose all sight in the eye. My eyes are fragile, that's the whole issue here. For those of you with connective tissue disorders, have you faced this same choice, to operate or not? What did you decide, and are you happy with the outcome? Was your doctor able to give you clear information to guide your decision? How many opinions did you seek out? Thanks in advance for sharing your experience.
7 Responses
177275 tn?1511755244
There are many eye complications in EDS. These include retinal detachment, lens subluxation, cataracts, glaucoma, dry eyes, macular problems. For more information read this article by the EDS Foundation http://www.ednf.org/medical-professionals/ehlers-danlos-syndrome-role-collagen-eye-0

I recently helped manage a patient with a collagen vascular problem (Marfan's syndrome). The best approach is usually a combined operation with a retina surgeon doing a vitrectomy, lensectomy, exam of retina and possibly endolaser. Then an anterior segment surgeon putting in an intraocular lens either an anterior chamber IOL or a sewed in posterior chamber IOL. After referring my patient to a University referral center they recommended an anterior chamber IOL   So you need a tertiary eye referral center with a team approach to fixing your eye. and likely two surgeons present for the operation a retina surgeon and an anterior segment surgeon.
1 Comments
Thank you, Dr. Hagan.
177275 tn?1511755244
Best of luck
Avatar universal
I have the hypermobility form of this disorder, generally considered benign, but it ain't necessarily so, and I value your advice re this little-understood condition.
177275 tn?1511755244
Agree not so benign
Avatar universal
it can a little startling when you see new doctors and mention EDS.  Sometimes they will say, 'Huh?'  Sometimes they gloss over it.  

A few years ago, I needed hand surgery for a trigger finger, and the plastic surgeon who did it was appalled when his supposed 20-minute job turned into 90 minutes.  Slightly wild-eyed, he told my husband that when he got inside my hand, there was nothing he could stitch anything to, because of the friabilty of the tissue.  He said it wasn't rheumatoid arthritis, but it was 'something.'  Ya think?  (I *had* told him beforehand.)  

This was my first confirmation of diagnosis by scalpel.
Avatar universal
It can a little startling when you see new doctors and mention EDS.  Sometimes they will say, 'Huh?'  Sometimes they gloss over it.  

A couple of years ago, I needed hand surgery for a trigger finger, and the plastic surgeon who did it was appalled when his supposed 20-minute job turned into 90 minutes.  Slightly wild-eyed, he told my husband that when he got inside my hand, there was nothing he could stitch anything to, because of the friabilty of the tissue.  He said it wasn't rheumatoid arthritis, but it was 'something.'  Ya think?  (I *had* told him beforehand.)  

This was my first confirmation of diagnosis by scalpel.
177275 tn?1511755244
That is why on the patient I posted with Marfan's the surgical team I referred her to decided not to do a posterior chamber sutured IOL because they did not want to put sutures in the sclera.
2 Comments
I am the original poster. I wanted to let Dr. Hagan know that I followed his advice, with a very good outcome.

I contacted the Marfans Foundation for referrals to doctors in my state who had experience with my specific set of issues. It turns out I did not have a subluxed lens, as suggested by one of my local doctors - instead I had a severe sudden-onset cataract, directly related to the surgery and vitrectomy which I had had done for my detached retina.

The specialist I found to do the surgery was a world-known expert in complex anterior cataract surgeries. I could not be more pleased with the results.It wasn't an easy process. I am grateful to the physician who worked with me and listened to all of my concerns, each step of the way.   I hope this outcome inspires others to forge ahead with their own care and advocacy.
I am pleased and happy for you and thanks for the follow up. My friend's daughter who has Marfan has had bilateral cataract surgery using a team of anterior and posterior chamber surgeons and while it was a long convalescence she also got excellent results.
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177275 tn?1511755244
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