Better and better…

· by · in day in, day out, idiopathic Ectopia Lentis, myopia (nearsighted), patching. ·

 My son Nicholas has ectopia lentis, a congenital condition that causes his natural lenses to move, or dislocate, from their normal position.  As a result we’ve had to manage high myopia, anisometropia, amblyopia, and the associated patching, glasses, replacement glasses, and even more replacement glasses since he was 6 months old.  A few months ago I posted about his surgery to remove the natural lens of his left eye and replace it with an intraocular lens.  Back with an update. 

Pleased to report that the healing / recovery throughout the intervening months has been uneventful.  The new lens is well centered, clear, and most importantly..providing good vision.

Prior to this surgery, N’s prescription has progressed to  -8.5 R and -23L and we worked diligently with his PO to ensure the patching routine was followed and we adhered to all follow up and recommendations.  This past week we were very pleased to experience the results of all the effort. 

After 4 years of prescription changes, extended patching, a surgery, some stiches here and there…and all the other challenges associated the PO shared with us the following…20/30 (with correction) bilaterally.  A first for us…ever.  Not only that, but patching time is reduced!   We go forward knowing the effort invested helped to maintain vision to the best possible given the circumstances, the right eye remains stable, and with a little luck and continued persistence his vision will remain the best it can possibly be.

21 responses to “Better and better…”

  1. Great news! So your son was 4 when he had his operation? I am asking because we were told about 4-5 years old when we could consider it.

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  2. What a great update, I’m so pleased to hear it. It must be wonderful to know that all that tme and effort (and glasses frames and repairs) have been worth it. How is Nicholas feeling about all this? He’s probably taken it in a stride as kids so often do.

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    • Ann,
      He’s not been too vocal about it. The PO informing him that he can wear the patch less was met with a sheepish smile and a grunt. He’s more interested in knowing when he can stop all together..but those questions are infrequent. I can say he’s very definitely aware of the improvement. Our routine is that he wears his patch to daycare and takes it off after lunch. In past with the vision at -23 he’d have to pull everything pretty close to view. Now, he’ll walk in and read the letter of the week on the bulleten board from across the room. Best is, the kids can go to a window and wave bye as their parents leave, before we’d have to come right up to the window for him to see…now he can track all the way across the parking lot…and waves and waves.. good stuff.

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  5. Thanks for sharing your story George. Our 3 year old was diagnosed with Ectopia Lentis et Pupillae 12 months ago and we have spent a lot of time researching and looking for other families going through the same thing. Finding this site was a revelation! I’m so pleased to read that Nicholas is doing so well an I’m keen to hear how he is now, 2 years later. Josh is scheduled for a lensectomy next year but no one can tell us with certainty whether or not it is the right thing to do and he will have to wear contacts instead of having an IOL inserted. We never imagined he’d wear the patches and glasses but now they’re just part of the daily routine so we’re hoping the contacts won’t be too traumatic. Thanks again, I’m feeling more optimistic after reading this!

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    • Hi, How is his vision presently? Have one or both of the lenses moved out of the visual axis? At 3 years of age a lensectomy would be the only option for most practitioners as there are very few who would do a lens implant at so young an age..

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      • Hi, his vision isn’t too bad (-10.5 and -7) and yes, both lenses are dislocated, although one more so than the other. We have the added complication that the vision in his right eye (the worst one) has actually improved with the patching but the lens has dislocated further and the zonules are very badly damaged. He would only have the right lens removed as it’s now crossing his field of vision (which is limited anyway as the pupil is also displaced!). We’re torn as none of the PO’s we’ve seen have been able to say with 100% certainty that surgery is the right option, all we know is that we need to do it soon to make the most of developing the neural pathways and maintaining the sight in that eye. I’m curious to know if Nicholas had the same issues with zonules and if so, how they attached the IOL?

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        • That’s the thing with EL and the zonules… one day progress, another setback. Nicky was similar, but reversed (-8R and -11L), and was fortunate in that the lens was for the most part in the field of vison. until the end. For as long as our PO was able to refract, we kept the lens in.

          Yes, same issues..progressive deterioration of the zonules. No PO will be able to tell you if it is right…but the one you go with should tell you it is the best option all things considered.

          Candid discussion with a PO you are comfortable with is paramount. Where the zonules are too far gone…there is the issue of preserving the capsular bag for future implantation of an IOL. Where it cannot be preserved the surgery is more complex. THis was the case with Nicholas. They could not preserve the capsule, and as a result..a scleral sutured IOL. They and he did however achieve excellent results.

          How does he see today… 20/?

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  6. Hi George, just wanted to thank you again for responding to our previous comments and give you a quick update. We had a very lengthy discussion with our PO this morning and have decided to go ahead with the lensectomy and IOL insertion next month. She’s opted for iris fixation due to the position of Josh’s dislocated pupil. We’ll still have to work pretty hard to correct the amblyopia but we should see a marked improvement in his vision (currently 20/36). Feeling pretty anxious but confident it’s the right thing to do. Happy new year to you and your family. Hope Nicholas continues to do well.

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  7. Hi George, just wanted to post a quick update. We had the first of two procedures yesterday and it didn’t go according to plan… They took the measurements needed for an IOL and found the Josh’s eye is small and flat, making fitting an IOL quite difficult. They also found that he would need one of 40 diopters and they simply don’t make them in that strength. The PO and a consultant she brought in were against the idea of an IOL to begin with so it’s given them a convenient reason to withdraw from the procedure. He’ll still have the lensectomy but will wear contacts unfortunately. We haven’t been given any other options so we’re going to try and get another opinion before the lensectomy, scheduled for 2 weeks time. They also cancelled an oblique recession (muscle surgery) at the last minute, fearing it would complicate things. We were really disappointed with the result and how it was handled, and Josh did not cope well with the anaesthetic so we’re dreading the next procedure… Just curious to know if you can recall the strength of Nicholas’s IOL, as his vision prior to surgery was similar to Josh’s?

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    • Hi Brooke,   Sorry to hear the procedure did not go according to plan.  It is a difficult and challenging process of measurement and calculation and estimation, both for strength (diopter) and size.  His age would also play a role as the eye may not be big enough yet for many types of IOL.  In reviewing your earlier posts, I see you had mentioned they were thinking of going with an iris fixated lens.  To be quite honest, this is quite murky territory for me, but based on my understanding overall such a lens would be placed in the anterior chamber (in front of the pupil).  Perhaps there may be options for a posterior placement (in the space where the lens was).  That is what Nicholas has.  A scleral sutured IOL.   Not sure the limitations at 3 years of age (N was 4 already, and the size of the IOL was not a concern.)  Am also not so sure about the limits of diopter range.  I know they can go as high as 40, but that is uncommon.  An appropriately sized IOL and diopter may not be available given the space available. (Nicholas, I think, had a 21 or 22 diopter lens placed)   My thoughts would be -to ask what consideration can be given to a posterior placement. (whether with this PO or another) It is a challenging surgery, so anticipate hesitation from whomever you speak with.  Perhaps a suitable diopter/lens could be found with this placement.  I think there would be considerable caveats though (an oversized lens may irritate, may not fit, and so forth.)   -to discuss contact lens vs wearing glasses.  Inserting and removing a cl at this age would be challenging from what I’ve read, but doable.  An aphakic spectacle would be thick, but would produce good results.  Remember, this would likely serve as an interim step.  As the eye grows, perhaps other options will present.   The anesthesia aspect of it is never easy.  Be brave, and focused on the goal..best possible outcome.   Best of luck.  Do keep in touch.      

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  8. Hi George,
    I am so glad I found this website and your post!
    My daughter is 4 years and 10 months old and recently diagnosed with ectopia lentis. SHe does not have lazy eye at this state. She can see 20/50 both eyes -corrected. uncorrected is probably over 20/200 both eyes. She is doing great with glasses -10.5 each eye.
    We tried contact lenses at first for one month. It was HARD! That one month was literally the worst month of my life I guess…
    We got extended contacts, and we would put them at night half an hour after she would go to sleep. That process was hectic . Some nights it would take us 4-5 hours to do that!!!!!
    And after all that hard work , she started getting allergies and discomfort with the lenses and dry eyes. We finally stopped it and got a glass for her.
    She is doing fine with the glass and she sees 20/50 -other kids her age see something around 2/40. so it’s not too bad…

    We have been told that surgery is not required yet and the lens has not moved in a year. so we’ll see how it goes in the future.
    I did some research for Artisan lens-Iris claw lens.It’s a European company and they have been using this aphakic lens all across the world for years but not in US.
    US is runnig a trial for this lens and they are accepting patients who would need implant for Artisan lens.
    please see the information here. i hope htis helps:
    http://clinicaltrials.gov/ct2/show/NCT01547442?term=artisan&rank=2

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    • Hi Amillia, Good to read that your daughter is tolerating the glasses and sees well. I am not to familiar with iris claw lenses. as I recall any issue with the iris would likely disqualify from that option. Ectopia lentis may be accompanied by instability of the iris (iridodonesis). We have had much success with IOL replacement to this point.

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  9. Thanks GOD for findingthis site…i am fron india my 5yr son who has febrile convulsion at age 1yr and from then on valparin syrup recently diagnosed with -10 &-11D.his eyesight too weak because of amblyopia .i hav vry worried because in our family no one wear glasses. Is it side effect of valparin or due to convulsion .however doctor refused about this

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  10. My name is Mike
    I am 56 years old and I was born with Ectopia lentis. I wore glasses that were an inch thick as a child. I went thru 12 years of school sat on the front row and never seen one thing on a chalk board or an overhead projector. I had 20/100 vision corrected. I had cataract surgery at 32 years of age and had 20/40L and 20/50R. I was woodworking one day and got a splinter in my right eye from a piece of African Wenge wood. The splinters are sepsis. Over the next year my cornea failed and I got a cornea ulcer on my eye. February of the following year I was in ICU for Afib and I felt a blister pop in my left eye. 6 months later my cornea failed in my left eye and my vision was CF in both eyes. I had a DSAEK in both eyes. I now wear a contact bandage Lens in both eye because of Filamentary Keratitis. I now have 20/300L and Cf in the right . I am so proud for you and Nicholas. Getting help at a young age is key especially if vision can’t be corrected with glasses. I wish everyone the very best. Never forget God is Gracious . He has blessed me so much.

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