Scleral strengthening in Marfan syndrome - Dr. Hagan?

[nickppatel]

Connective tissue weakness appears to be at play in severe myopia in Marfan patients.  As you may be aware, there has been enormous excitement in the Marfan community lately based on research originating at Johns Hopkins using ARBs (and in some studies, ACEi's) like losartan to reduce TGF-b signalling and thus arresting or even reversing aortic dilatation.  

Since it's probable that the same underlying signalling issues are at play with Marfan-based myopia as with aortic dilatation, would you venture a guess on whether these drugs might also stop myopic progression and strengthen the sclera?  I understand that the eyes are very rich in fibrillin.  

I have some sort of connective tissue problem (not Marfan, but perhaps MASS syndrome), and I began using ARBs (valsartan) a few years ago, when this research first debuted.  I graduated to a maximal dose (320mg/valsartan/day) about a year and a half ago.  I don't know if this is wishful thinking or what, but my myopic progression and aortic dilatation both seem to have completely stopped (and aortic dilatation actually appears to be reversing to normal size).  But perhaps the myopia is just halted for the time being, or perhaps it's stopped just because I'm now 28 and it's not going to progress further.  

So, in summary, even if you don't know for sure, any guesses would be fantastic ... particularly whether you know if upregulated TGF-b signalling is responsible for scleral stretching in Marfan syndrome.

Thanks!

[John C Hagan III, MD, FACS, FAAO]

I am sorry but I do not think I can render a valid opinion on this type of research. Perhaps a Marfan support group might have that information.

JCH MD

[Cameron12321]

Hello Sir,
I am 21 years old highly myopic since birth with -17 in left and -23in right last year had a retinal detachment in right and lost some peripheral vision which  scared me a lot and I am seeing a psychiatrist to overcome my anxiety , no one in my family has high myopia
  yesterday I came across the term marfan syndrome on internet my question is should I ask my family doctor about this because apart from high myopia I have other symptoms like tall thin body ,long fingers ,hands and positive wrist sign test .
Otherwise I have no other problem as I am very active in sports , should I be concerned or I am getting over anxious ?

[JohnHaganMD]

Yes I would ask your family physician about Marfan's and ask your ophthalmologist if there is any suggestion of dislocation of the natural lens.

[Cameron12321]

Hello Sir
I told my mother about this, apparently she did have high myopia-10  in both eyes and had LASIK before marriage which she never told me about , then I showed her the wrist sign she said it is because I am thin and had. lost   weight due to my anxiety , and told not to worry, what should I do , should I see my physician?

[Cameron12321]

What other reasons could be of my myopia or the wrist test ?

[JohnHaganMD]

the genetics of myopia are incompletely understood but if your mother was -10 myope that alone explains why you are high myope.  Marfan's often runs in families if no one has it it is less likely you have it.  You would probably get some peace of mind by seeing your family physician or pediatrician and find out for sure you do not have Marfan's

[Cameron12321]

Thank you so much Sir for your reply

[JohnHaganMD]

Sure.

[Cameron12321]

Hello Sir,
I want to know if anything can be done to prevent RD or other retina problems in my good eye? I am worried about my left eye,  I have 20/20 vision acuity in left but right eye doesn't have good acuity due to myopic degeneration and is ambylopic?

[JohnHaganMD]

Yes to reduce risk of RD.  wear impact resistant lens in your glasses and sturdy frames; avoid high impact sports such as American football, rugby, soccer, wrestling, etc and avoid racquet sports (tennis, squash, racket ball), if you do wish to take the risk of participation wear sports specific eye protetion. Avoid amusement park type rides like roller-coasters that whip head about. Have your eyes examined annually by an Eye MD ophthalmologist and live a healthy lifestyle, don't use nicotine or cannabis products, if you don't use alcohol don't start, if you do no more than one drink per day.  Eat a good diet and exercise things like walking or swimming.