ICE Syndrome, Sturge-Weber Syndrome, Inflammatory & Traumatic Glaucoma
Chat Highlights
December 12, 2001

Norma Devine, Editor

On Wednesday, December 12, 2001, Dr. Rick Wilson, a glaucoma specialist at Wills, and the glaucoma chat group discussed "ICE Syndrome, Sturge-Weber Syndrome, Inflammatory & Traumatic Glaucoma."

Moderator:  Doctor Rick, tonight we would like to discuss four types of glaucoma:  Iridocorneal endothelial (ICE) syndrome, Sturge-Weber syndrome, inflammatory glaucoma, and traumatic glaucoma.  What do these types of glaucoma have in common?  

Dr. Rick Wilson:  They are all secondary glaucomas, caused by a visible problem, as opposed to chronic open-angle glaucoma, the cause of which is unknown.  In ICE, a spectrum of diseased corneal endothelium, the inside lining of the cornea, grows off the cornea, covers the drain or pulls the iris up over the drain, blocking it.  The cornea is injured by this abnormal lining and often gets swollen at a lower-than-usual intraocular pressure.  

Moderator:  What causes ICE?  

Dr. Rick Wilson:  ICE may result from a herpes simplex infection of the cornea in utero (before birth).

P:  At what age does ICE commonly occur?  Is it unilateral?

Dr. Rick Wilson:  The ICE syndrome is usually seen in women in the 30 to 40 age group on diagnosis, and affects only one eye seriously.  A few changes can be seen in the other cornea, but that eye typically remains healthy.  Does anyone in the room have one of the four types of glaucoma we're discussing?

P:  I have traumatic-angle recession mixed with pigmentary.  It's been hard to diagnose.  I have a big problem with intraocular pressure.

Dr. Rick Wilson:  Your iris may have been knocked posteriorly (toward the back of the eye).  The iris could be rubbing on the ligaments that hold the lens in place, knocking off the pigment on the back of the iris.

P:  I have traumatic-angle glaucoma. 

Dr. Rick Wilson:  Angle recession happens when the eye is hit hard, sending a fluid wave posteriorly that tears the iris away from its usual insertion into the wall of the eye.  Examination with a gonioscope reveals the damaged iris root.  The injury also hurts the trabecular meshwork (the drain of the eye), which then no longer works as well.

P:  Does angle recession mean the angle is smaller after the injury?

Dr. Rick Wilson:  No, the angle is usually larger since the iris has been pushed posteriorly permanently, although to a small extent in most patients.

P:  Would laser surgery help such a problem?  

Dr. Rick Wilson:  No.  Laser surgery is not usually helpful in the secondary (having a known cause) glaucomas, unless the angle is narrow.

P:  What causes Sturge-Weber syndrome?

Dr. Rick Wilson:  Sturge-Weber is caused by a vascular abnormality called a port wine stain, a red blotch of vessels under the skin, usually on one side of the face.  If the upper lid is involved, usually there are vessels growing on the surface of the globe, which have a higher blood pressure than normal.  That makes it harder for fluid to leave the eye.

P:  Are inflammatory glaucomas associated with other inflammatory diseases, such as rheumatoid arthritis or juvenile rheumatoid arthritis?  

Dr. Rick Wilson:  Yes, inflammatory glaucomas can be associated with juvenile rheumatoid arthritis or rheumatoid arthritis or other systemic inflammatory conditions.

P:  Can the damage caused by trauma to the eye take years to become evident?  

Dr. Rick Wilson:  After trauma to the eye, glaucoma may take years to surface because the eye's natural excess capacity for fluid outflow is prematurely lost.

P:  My glaucoma took 27 years to surface.

Moderator:  Is that a long time for traumatic glaucoma to surface?  

Dr. Rick Wilson:  Not really. 

P:  Mine took about 15 years to surface.

P:  What surgery or medications would help?  

Dr. Rick Wilson:  Usually medicines designed to decrease the amount of fluid the eye makes are most effective.   Surgery is also usually successful, but not as much as with chronic open- or closed-angle glaucoma, and the surgery has more frequent complications. 

P:  Do shingles (herpes zoster) on the face cause the port wine stain?  

Dr. Rick Wilson:  No, that is a birthmark.  

P:  Would you see the "evidence" of the trauma later, or might it never be clear if the problem was from trauma or some other type of glaucoma?   

Dr. Rick Wilson:  If there is serious trauma, there should be some microscopic evidence that could be picked up.  Moderate trauma may not leave a lasting scar, so then the clue would be a one-eyed glaucoma that may come on more rapidly than a garden-variety, chronic open-angle glaucoma.

P:  Can the trauma to the eye be caused at birth by forceps?  

Dr. Rick Wilson:  I have seen corneal injury from forceps, but so far not a traumatic glaucoma.

P:  I had an eye badly damaged at birth by forceps.  It cleared up fairly soon, but could that have caused glaucoma?

Dr. Rick Wilson:  If the forceps injured both eyes, but that would be unusual. 

P:  How do you differentially diagnose inflammatory glaucoma versus primary open angle?

Dr. Rick Wilson:  Inflammation in the eye causes the part of the eye that makes the watery fluid to also leak serum protein from the inflamed blood vessels.  When a beam of light is shown through the anterior chamber of the eye, the beam stands out like a beam of sunlight in a smoky room.

Moderator:  Can a chemical burn in the eye lead to traumatic glaucoma?

Dr. Rick Wilson:  Absolutely, especially basic, more than acid, chemical burns.

P:  If a patient's intraocular pressure (IOP) is elevated due to taking steroids for inflammatory glaucoma (in addition to high IOP caused by the inflammation itself), and glaucoma surgery is performed, how can one ensure that hypotony will not occur if the steroids are eventually withdrawn?

Dr. Rick Wilson:  In a shunt, a tube keeps the hole open no matter how much fluid pushes its way through.  If the eye does not make a normal amount of fluid, the shunt will continue to drain a normal amount and the intraocular pressure will be too low.  But with a trabeculectomy, the hole is not held open and will close down to whatever amount of fluid flows through the tiny tunnel through the sclera.  Too low a pressure is usually a problem only if mitomycin-C or 5-FU keeps the hole from healing down normally.

P:  Does that apply to both valved and non-valved shunts?

Dr. Rick Wilson:  Yes, the valves don't work too reliably.

P:  My daughter has secondary glaucoma caused by uveitis.  Once she stops using steroids, will her eye pressures decrease?  She's using prednisone and Pred Forte eye drops. 

Dr. Rick Wilson:  Probably 95% of chronic open-angle glaucoma patients are steroid-responders, which means taking steroids (usually topically) raises their eye pressure. On the other hand, the percentage of steroid responders among the inflammatory glaucoma population should be much closer to the general population, which is five percent at six weeks.  It increases with time.  That is a long-winded answer to say your daughter's IOPs may or may not decrease.  I can't tell from the history you have given me.  

P:  She had Baerveldt implants this year, and has had uveitis for two years.

Dr. Rick Wilson:  The shunts should allow her to take steroids without as much worry about the steroids increasing the IOP.  

P:  Is chronic open-angle glaucoma primary or secondary?  Which one is more serious?

Dr. Rick Wilson:  Chronic open-angle glaucoma can be primary or secondary The secondary glaucomas are usually worse, but a few are curable.

Moderator:  Thanks again, Dr. Rick. 

Dr. Rick Wilson:  Everybody have a good week.  See you here next Wednesday night.

End of highlights for December 19, 2001.

On December 19, Dr. Wilson discussed "Pseudoexfoliative and Pigmentary Glaucoma" in the Chat room. Click here for highlights of that meeting.

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