The Letter With the Details

Dr. Trese in Michigan dictated a letter to our doctor at Duke yesterday.   I tried to take notes, but he said so many large words so quickly that I gave up.   I think I would have gotten a lot of it but I was typing on an iPad with my laptop out of commission and autocorrect was working against me, correcting technical terms I was likely misspelling into normal, not-medical words that weren’t at all what I was trying to write.   Today, I got a copy of that letter and now I can report exactly what’s going on and what the plan is.

We had the privilege of examine your patient on 12/14/2015.   Certainly, she has a very complicated history including workup for immunologically derived bilateral serous detachments.   

My understanding is that the child failed their school screening in March of this year and started to show significant visual reduction in August of this year with an episode of bilateral erythema followed by discovery of bilateral serous retinal detachments which appear to have progressed to a tractional detachment with hypotony in each eye.  The traction appears to be involving a large amount of subretinal fibrosis, seemingly with minimally visible epiretinal tissue at this point in time.   The child does have light perception in each eye, with better form vision in the left eye. 

We did do an exam under anesthesia with water bath OCT and B-scan.  We found a spherophakic lens and intraocular pressure of essentially 0 in both eyes.  She does show significant amounts of uveal entropion in each eye and I think certainly manifests changes of an anterior dysgenesis circumstance. I think that she may also have a vitreous retinal dystrophy as well which may have been part of the evolution from serous to what appears to be now predominately tractional posterior retinal detachment.   In her better left eye, there appears to be areas of avascular retina or bare choroid in some areas which we cannot be sure of but her visual function is undoubtedly better in the left eye.   The exam of the right eye shows areas of retina that do appear to still have small amount of sub retinal fluid and vessels that extend further towards the periphery.  I think that more likely than not this represents the anterior and posterior ocular dystrophy.  I am not sure that this is a named ocular dystrophy but I do think that the hypotny which currently allows the child to have clear corneas may also have contributed to an even larger reduction in globe size.  Her corneal diameters being approximately 7 and 8 mm in horizontal diameter.  I think this also represents part of her anterior segment dysgenesis.  

We have talked to the family about surgical intervention in her worse right eye which is just light perception.  Our plan is to try and leave the spherophakic lens in place, realizing that it may have weakened zonules and to proceed with vitrectomy and silicone oil with perhaps Healon injection in the anterior segment.  

I will keep you posted as to her progress and thank you very much for the privilege of sharing in her care. 

Let me know when you figure out what all that means.   We’re still working on it here.