Inside My Daughter's Eyes

Alright, I'm going to try and explain what's happening inside my daughter's eyes.   I'm at the business center at our hotel at a real monitor and keyboard and I'm able to think and type while my husband and daughter are in our room getting ready to check out. 

Dr. Trese said she was one of the most complicated cases he's seen in a while.  He said trying to piece everything that's going on inside her eyes into one diagnosis would be nice, but it may not be possible.  He said at one point in a casual, almost offhanded way, "I get all the crazy cases".   And that's exactly why we went to see him.  We need an expert on crazy.  

As he talked, I took notes as fast as I could and I'm going to try and explain it, but I don't know that I fully understand it myself so this information may be updated in a later post if and when we learn more.

She has extensive sub-retinal fibrosis.   This is contributing to those crazy folds you see in her retina.   What happened, he thinks is the serous retinal detachment caused fluid to build up behind the retina.   Those cells turned into scar tissue.   The serous detachment is resolved at this point, but that serous fluid was a culture medium for scar tissue to grow.  That scar tissue has now contracted.   The shrinking has caused the folds in her retina to worsen.   

This means the retinal folds can't lie back down onto the choroid because they're being held in the folded mess.   This means the fovea centralis, or central focal point we use for our main vision is folded and can't get information from the lens.

What caused this?  Dr. Trese said he tried to look up something he rembers from about fifteen years ago while he was in the OR but he couldn't find it.   There was a case of multiple people who had something similar that was triggered by a viral infection.    He's going to see if he can find it for reference prior to our meeting today.

As far as her eye in general go.   They are not correctly formed.   The lenses are almost round.   Lenses are not as thick as they are wide.   Her lenses are five millimeters thick, making them almost spherical.   Both eyes are smaller by a significant amount.   The corneas diameter measures 7-8 and should be 10-11.  These are things she was born with.   Did they contribute to her current situation?  Was she pre-disposed to something triggering her current condition?  The answers to these questions are unknown.  There is evidence that conditions like hers are connected with virteal detachment.

The pressure in her eyes is at zero, which is very low and is not good.  The right eye appears to have abnormal circulation.   Circulation in the left eye looks worse than the right eye.   It appears the left eye may have had a very large retinal tear as there is no evidence of blood flow in that part of the retina.  This is an indication those cells have died and zero information is being transmitted in that area.  The center part of both eyes is terribly folded.

Is there a leaking of fluid?  I had talked about the fluid producing cells and a possible leak in a prior post.  With central retinal dystrophy there is an association with colalgen issues, which could be an important clue.  Sometimes children who have this can have exposed areas of the choroid causing a leaking of fluid. 

Those fluid producing cells are called cillary.   The cillary body, which is found in front of the retinal area, looks atrophic.  This means either there is decreased production or an increased outflow.   This is still an unknown.  There appears to have or have been fluid behind the cillary area similar to the serous detachment, further causing complication in her eyes. 

There is an effluvia effusion syndrome that might account for what has happened, especially since it happened bilaterally (to both eyes).   Was it a viral infection?  If she didn't have the vitreous retinal dystrophy it might not have resulted in the damage to her eyes. 

Dr. Trese is not sure if there is any genetic testing to be done related to the speherical lenses but he is going to look into it. 

What can be done to help stop the process of vision loss, return the pressure to her eyes and stabalize her vision?   He said she was one of three of the most complicated cases he's seen in a while and making a plan to help while not causing more harm or reduction in her remaining vision is important. 

The low pressure can be address with the addition of silicone oil into the eyes.  The oil will remain and won't permeate out.   This has long-term issues such as causing cataracts, but lenses can be replaced with artifical lenses later.   The best plan would be to inject the eye she is using less (right eye) and see how that changes her vision.  If there is a positive outcome from that then proceede with the left eye. 

There are other ways to increase the pressure without silicone oil but they require continual injection of a semi-solid substance called Helon.   He doesn't want to do anything that increases her risk, but he doesn't want to lose more of her vision either. 

Today we return to Dr. Trese's office for him to evaluate what she can see now by observation.   We may return next Monday or early in January for an injection in one of her eyes.