Chat Highlights
Glaucoma and the Young
June 20, 2001

Norma Devine, Editor

On Wednesday, June 20, 2001, Dr. George Spaeth, a glaucoma specialist at Wills, and the glaucoma chat group discussed "Glaucoma and the Young." 

Moderator:  Welcome, Dr. Spaeth. 

Dr.George Spaeth:   Thank you.

P:  I was just saying that you performed one of my cataract operations in 1973.

Dr. George Spaeth:  That's a lie.  I was still in kindergarten.

P:   I see you have not lost your sense of humor.

Moderator:  Dr. Spaeth, the topic is Glaucoma and the Young. 

Dr. George Spaeth:  Unfortunately, glaucoma does occur in the young, and it is usually a serious problem. 

P:  I am the first known case of glaucoma in my family tree.  What are the risks for my sons and their children?

Dr. George Spaeth:  Okay, a bit about genetics.  There are lots of different ways glaucoma is inherited.  The risk for children whose parents have ordinary open-angle glaucoma is five percent. 

Moderator:  Dr. Spaeth, are there certain types of glaucoma seen in the young or many types?

Dr. George Spaeth:  There are many types.  Congenital glaucoma, for instance, is glaucoma that occurs at birth or shortly afterwards.  It's rare and usually does fairly well with surgery, if surgery is done by somebody good, like Dr. Wilson, and promptly.  The commonest type of glaucoma in the young is probably secondary to trauma.

P:  I am the first case of Axenfeld-Reiger syndrome in my family.  I was diagnosed when I was eleven years old.  How worried should I be for my children, who are now now 13, 10 and 5 years old?

Dr. George Spaeth:  The inheritance probability of  that syndrome has been worked out fairly well.  I don't know it off the top of my head.  A trip to one of the standard texts would give the answer. 

P:  I was born with glaucoma.

Dr. George Spaeth:  How did yours do?

P:  I'm now 31 years old and doing well, but I do have these lines on my cornea.

Dr. George Spaeth:  Those lines have a fancy name,  Haab's striae.  They occurred because your eye got stretched and pulled apart at the back side of the cornea. 

Moderator:  What are Haab's striae?  

Dr. George Spaeth:  Breaks or tears in the cornea.

P:  How likely are my corneas to fail?

Dr. George Spaeth:  Not likely.  Your corneas will probably hold out well. 

P:  Could I have a cornea transplant?  

Dr. George Spaeth:  If you need it in the future,

but it's not likely.

P:  If I ever need cataract surgery, can that be done on me?  

Dr. George Spaeth:  If you need it, sure.

P:   How soon after birth is considered "congenital?"

Dr. George Spaeth:  "Congenital" means that it has already started at the time of birth.

Moderator:  Does trauma often result in (relatively) immediate glaucoma in the young, or is it more usual for it to take a long time to show up?

Dr. George Spaeth:  Trauma has two phases.  Immediate glaucoma, which is especially serious in people with sickle cell disease, and much later, literally not showing up for about 20 years.

P:  What glaucoma symptoms are noticeable in a child?  It would be rare to have a pediatrician check IOP (intraocular pressure).

Dr. George Spaeth:  The symptoms are light sensitivity, tearing, and an eye that doesn't look shiny and clear. Then the eye gets bigger.

Moderator:  Why does it get bigger?

P:  Does the whole eye get bigger or just the pupil?

Dr. George Spaeth:  The eye gets bigger because it gets stretched by the high pressure. Glaucoma in infants is always the result of high pressure.  The pupil does not get bigger; the entire eye does.  A good way to tell how the child is doing is to measure the length of the eye, and if it is getting longer, the pressure is probably too high.  

P:  My healthy 11-year old daughter has excellent vision and a normal IOP of 16 mm Hg.  She has been told to have a visual field test and an HRT (Heidelberg Retinal Tomography).  Her cup-to-disc ratio is off, which may be an early indicator. What does that mean? What else would cause a .6/.5 and .8/.6 ratio?  How concerned should I be?   There is no known family history of glaucoma, but there is macular degeneration and retinitis pigmentosa on the maternal side.

Dr. George Spaeth:  Discs that have "big cups" tend to be thought of as glaucomatous, but they may just be big discs.  Low-tension glaucoma in children is rare.  I mean really rare, not likely.    

P:  What would be the follow-up after initial testing?  Annual exams, or more frequently?  If my daughter has big cups, will her vision ever be affected?

Dr. George Spaeth:  The most important thing is getting baseline photos of the discs and visual field tests.  (If I were the health czar,  I would get disc photos of everybody at age 15 and archive them.  That would be a fantastic help.) If the discs are normal, I would probably recommend  a yearly follow-up.  Myopes (near-sighted people) have big eyes and tend to have big discs.  Big discs have big cups.  Is your daughter more near-sighted in the eye with the bigger cup? That is routine. 

P:  Actually her vision is a bit far-sighted in both eyes. Could you tell me what the number in the cup-to-disc ratio means?  

Dr. George Spaeth:  Think of the disc as a doughnut and the cup as the hole in the doughnut.  A  ratio of  .5 means half the disc is cupped.

P:  What is 'notching' as an abnormality of glaucoma?

Dr. George Spaeth:  A notch is a notch in the doughnut portion of the disc, as if somebody took a bite from the inside.

P:  I'm confused about the notch.  

Dr. George Spaeth:  Picture the doughnut.  Now concentrate on the inside rim of the doughnut.   It is usually round.  But now picture the doughnut as very thin in one place because the inner rim is not round, but gouges into the doughnut in one local area.

Moderator:  So notching indicates optic nerve damage?

Dr. George Spaeth:  Yes.  A notch is always a sign of damage.  That is why it is important.

P:  Is the cup-to-disc ratio (.6/.5 and .8/.6)  really significant numerically for my 11-year-old daughter?   

Dr. George Spaeth:  Everybody is unique and everybody shares common things with everybody else.  But one person can have a moderate cup that is damaged or healthy, while another person can have a big cup (.8) that is damaged or healthy.  It all depends on the size of the cup your daughter started with. 

P:  Well, as of now, the numbers are the first and just routine measurements. So I guess we have to say that's where her size is starting off.  She will have the Heidelberg Retinal Tomography and go from there. 

Dr. George Spaeth:  The HRT is a good idea.  

P:  I have a December birthday girl, so she is the youngest in her class.  Should she be kept back one grade because she will have visual impairment from her open-angle glaucoma?  

Dr. George Spaeth:  I would not change her school plans yet.

P:   Won't her reading potential be affected sooner or later? 

Dr. George Spaeth:  That's an important question I can't answer.  Does she have any visual impairment now?

P:  Not yet, but she has had two trabeculectomies, a goniotomy, and next maybe the Ahmed  filter.  Wouldn't changing school plans in the future be a harder adjustment, with also adjusting to vision loss? 

Dr. George Spaeth:  I don't think so.  A good rule is to keep the children doing "normal" things as long as they can, perhaps forever.

P:  How do you feel about using the Ahmed valve in children?

Dr. George Spaeth:  The Ahmed can work well.  I have used it in kids with success. But glaucoma in kids is tough. The Ahmed is probably no better or worse than the Baerveldt implant.  

P:  Are the treatments for children the same as for adults:  drops to reduce pressure, then surgery as a last resort?  Or is surgery an earlier option for the young?

Dr. George Spaeth:  No, the treatment for kids is very different.  Most need surgery.  Drops  can be dangerous in kids.  Beta blockers can make breathing hard, and Alphagan can affect  brain function.  An interesting difference between glaucoma in kids and adults is that in adults the pressure doesn't need to be high to cause damage, but in kids it does need to be high.

P:  If Alphagan is known to affect the brain function in children, what kind of effect does it have on adults?

Dr. George Spaeth:  Alphagan also affects adults.  It  makes them tired.

P:  I'm 16 years old, and have no family history of glaucoma.  Ever since I was 13 I have been found to have an IOP of 21 mm Hg to 28 mm Hg and a "borderline" retinal picture. What do you think my chance is for developing glaucoma?

Dr. George Spaeth:  With a pressure of around 25 mm Hg,  given no other risk factors, your chance of developing glaucoma is about 10 percent.  However, you are young and so you have a long way to go.  

P:  Once a normal IOP has been established after a trabeculotomy, at what point does the risk of a relapse diminish?  In other words, how long before the child is "out of the woods?"

Dr. George Spaeth:  The child is never out of the woods.  Trabeculectomies tend to fail about 15 to 20 years later.  Early on, trabeculotomies work pretty well for a long time, once it is clear that they are working.  They may fail within a month or so, but if they are working after that, the outlook is good.  

P:  If a parent has glaucoma, how often should the children be checked if their eyes are normal at birth?    

Dr. George Spaeth:  That depends on the type of glaucoma.  If it's ordinary primary open-angle glaucoma, the children don't need to be checked more carefully than any other children.   The danger starts around age 45 or so.  If  parents got glaucoma when they were young, then their kids need checking early, maybe around 15. 

Moderator:  Do you think that people born today with glaucoma will see a cure in their lifetime?  

Dr. George Spaeth:  Glaucoma is 100 or so diseases;  some of those diseases will be cured; some may never be cured. 

Moderator:  If only there were a pill to make us healthy for life.  

Dr. George Spaeth:  If there were a pill to make people healthy, no one would ever be healthy. Health only comes from within.  It is nothing that any doctor or any pill can provide.  

P:  In a young person, a blood injection for hypotony caused by mitomycin C seems like a temporary solution, since IOP decreases again with time.  

Dr. George Spaeth:  Any hypotony is tough to solve.  There's not much difference there between kids and adults.  A blood injection is often temporary.  More definitive surgery may be needed. 

P:  The only good news about having glaucoma is that those afflicted are universally good-looking, intelligent, and fun at parties.

Dr. George Spaeth:  Can you cite me a reference (other than personal)?

P:  I thought that it was too evident to have a study.  Sort of like studying why the sun rises in the east.

Dr. George Spaeth:  I liked the questions about the disc.  That is where we have to pay attention. 

P:  Dr. Spaeth, clearly we need to be better informed about the optic disc, the effect of the nerve size on the cup-to-disc ratio, the significance of a notch, whether less attention is being placed on the size of the cup, etc.  Could you join us sometime for that discussion? 

Dr. George Spaeth:  Yes, I'd be happy to do that.

P:   Excellent job, Doctor.  Thank you for coming.

Moderator:   Thank you for your time, Dr. Spaeth.   Good night.

End of chat highlights for June 20, 2001.

On June 27, Dr. Wilson discussed "Genetics and Glaucoma" in the Chat room. Click here for highlights of that meeting.

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