ICE Syndrome
Chat Highlights
July 23, 2003

Norma Devine, Editor

On Wednesday, July 2, 2003, Dr. Rick Wilson, a glaucoma specialist at Wills, and the glaucoma chat group discussed "ICE Syndrome."

Moderator:  Dr. Wilson, tonight's topic is iridio-corneal syndrome, commonly referred to as the ICE syndrome.  A couple of patients here tonight have the ICE syndrome.  What causes it? 

Dr. Rick Wilson:  Glad to have them here.  The ICE syndrome is caused by the diseased lining of the cornea, which grows over the drain in the eye, blocking it, and over the iris, causing stretching and a lack of blood supply.

(Editor's note:  ICE syndrome is actually a grouping of three closely linked conditions:  iris nevus (or Cogan-Reese) syndrome; Chandler's syndrome; and essential (progressive) iris atrophy, which accounts for the acronym ICE.)

P:  One hypothesis is that ICE has a viral origin, an in-vitro herpes infection localized in the endothelial layer.  Does that mean the herpes infection is responsible for allowing the endothelial cells to remain capable of multiplying and migrating?

Dr. Rick Wilson:  We still do not know how the herpes virus causes the ICE Syndrome.  All we know is that a high percentage of corneal graft specimens from ICE patients undergoing keratoplasty contain the herpes simplex virus.

P:  What happens if my friend's graft fails?  

Dr. Rick Wilson:  Your friend's cornea is cloudy.  If the clear corneal graft turns cloudy, she will be the same as before.  

P:  With just a small amount of cloudiness on the cornea and without a graft, how long will my friend's vision last?  

Dr. Rick Wilson:  Sometimes for years, often less.  It's quite variable.   

P:  Are peripheral anterior synechiae and the abnormal membrane that grows over the back of the cornea in Chandler's syndrome the same or different conditions?  

Dr. Rick Wilson:  The diseased lining that grows over the iris shrinks with time and pulls the iris back up toward the cornea, covering parts of the trabecular meshwork (drain) and closing it off.  The membranes are one and the same.

P:  Since the herpes virus may be the cause of ICE, are you trying Valtrex on any of your ICE patients? 

Dr. Rick Wilson:  Not yet.  Since an ICE patient would need to use Valtrex for years, I would first want to know that it really worked.  

P:  Would there be a serious side effect from using Valtrex over the years?

Dr. Rick Wilson:  I can't answer that.  Perhaps a corneal specialist could answer,  if  there's enough experience with chronic therapy for years to know the answer.

P:  Will you please define disseminated ICE and total ICE?

Dr. Rick Wilson:  Sorry, I don't know those terms.  The terms we use are iridocorneal endothelial syndrome,  Cogan Reese, progressive iris atrophy, Chandler's, iris nevus syndrome, and essential iris atrophy.

Moderator:  Are those basically the same thing?

Dr. Rick Wilson:  Those terms all describe the different parts of the spectrum that is ICE.

P:  Does damage caused by glaucoma or damage to the cornea pose the greater threat to ICE patients?  

Dr. Rick Wilson:  The cornea can often be repaired with a graft, so the threat to the optic nerve from the glaucoma is the greatest concern for me.

P:  Does the appearance of the iris change in Chandler's?

Dr. Rick Wilson:  Patients can start out with few signs except in the cornea.  Over the years, the iris begins to show signs such as Cogan Reese, where small bumps appear on the peripheral iris.  Sometimes Chandler's does not change for many years.

P:  I mean is the iris change visible to the naked eye, as with essential iris atrophy?  

Dr. Rick Wilson:  Occasionally, although it takes years to happen and may not happen at all.

P:  Is warm air blown on the face with a hair drier used to speed up dehydration of the cornea in ICE, especially in the morning?

Dr. Rick Wilson:  Only if there is corneal edema (swelling) that the hairdryer can dry out and make the cornea less swollen, and therefore clearer.

P:  In searching for abstracts on ICE syndrome, I have found only retrospective studies on a small number of patients.  Are you aware of any prospective studies?  Is the ICE glaucoma population too small to justify large studies?

Dr. Rick Wilson:  It is difficult to do prospective studies when the number of patients at each center is so small.  It would take many centers and a good many years for a prospective controlled trial, such as one for Valtrex.

P:  Is ICE related to POAG (primary open-angle glaucoma) or NTG (normal-tension glaucoma) or any other distinct diseases we know of?

Dr. Rick Wilson:  No, other than herpes.

P:  Are the causes of  failure of trabeculectomies in eyes with ICE the same as for eyes with other types of glaucoma?  Does the type of glaucoma predict the way in which a trabeculectomy will fail?

Dr. Rick Wilson:  Yes, it does.  Trabeculectomies in patients with ICE may work beautifully for many years, then be over-run by the corneal membrane, or may fail after just a few months when they were working quite well.  The membrane can go through the man-made hole in the eye and line the inside of the bleb.  Such patients present with high blebs and high pressures.

P:  What about tube shunts? 

Dr. Rick Wilson:  The membrane has a hard time climbing up the tube, so aqueous (tube) shunts often work better for longer than trabeculectomies.  Shunts, however, have a poorer prognosis for the graft, if one is needed, than for the trabeculectomy.

P:  Is it better to have the transplant to prevent damage?

Dr. Rick Wilson:  Not unless you need it.  The graft requires a year of drops and many visits to the corneal specialist.  That's not something to do unless your vision forces it on you. 

P:  Have you ever seen an ICE patient in which ICE attacked both eyes? Are there any theories about why it is unilateral?

Dr. Rick Wilson:  I have seen one of two patients where there seemed to be just a touch of ICE in the other eye, but it did not progress.  I have seen many people, including a seven-year-old girl, who seemed to have ICE in both eyes, but showed no progression after years.

P:  Is it known why only one eye is usually affected? 

Dr. Rick Wilson:  The reasoning feeds into the herpes theory.  In that theory, one eye is infected first and immunity develops before the second eye can be affected.

Moderator:  Dr. Wilson,  just so people understand clearly,  glaucoma due to ICE is a secondary glaucoma, and is not considered POAG (primary open-angle glaucoma), correct?

Dr. Rick Wilson:  That is correct.  It is a secondary angle-closure glaucoma.

P:  An irregular pupil is a characteristic of ICE. Why does the iris get pulled out of shape?

Dr. Rick Wilson:  As the membrane contracts, it pulls the iris up over the drain and can also pull the iris apart.

P:  Is damage to the iris permanent?

Dr. Rick Wilson:  Yes, the iris does not heal.  That is why an iridectomy (a hole made with a laser) will stay open for life.

P:  Do most patients with ICE who develop glaucoma get closed-angle glaucoma?

Dr. Rick Wilson:  Yes, either corneal membrane or iris covers the angle.

P:  I have a tube shunt and Chandler's.  My doctor said I will need to massage my eye occasionally to clean the debris out of the shunt. Could you please explain that to me?  

Dr. Rick Wilson:  Some surgeons believe that pushing on the cornea will force aqueous fluid through the shunt and dislodge any debris forming in the shunt, or in the pores in the scar tissue surrounding the plate.

End of highlights for July 23, 2003.

On July 30, Dr. Wilson discussed "Testing Equipment" in the Chat room. Click here for highlights of that meeting.

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