Young Glaucoma Patients
Chat Highlights
June 30, 2004

Norma Devine, Editor

On Wednesday, June 30, 2004, Dr. Elliot Werner, a glaucoma specialist at Wills, and the glaucoma chat group discussed "Young Glaucoma Patients."

Moderator:  Dr. Werner, do you treat many younger patients?  

Dr. Elliot Werner:  That depends upon how you define young.  Since I am 58-years old, anybody under 60 is young.  Generally, however, the term "juvenile glaucoma" usually refers to people under 30 years of age.  Most glaucoma patients are, of course, older.  Glaucoma in younger patients is relatively uncommon, but not rare.  It is common enough that we glaucoma specialists will see a moderate number of younger patients.

P:  Do most of the younger patients have primary or secondary glaucoma?

Dr. Elliot Werner:  In my experience, it's about 50-50.

P:  What are trabeculodysgenesis and iridotrabeculodysgenesis?

Dr. Elliot Werner:  Wow, what a way to start.  Those terms refer to a congenital (present at birth) anomaly of the structures in the angle of the eye.  The structures fail to develop properly during embryonic life; therefore, they do not function well in later life.

P:  Does congenital glaucoma mean it was present at birth? 

Dr. Elliot Werner:  Yes, the term implies "present at birth," or at least the abnormality causing the glaucoma is present at birth.  Any apparently open-angle primary glaucoma that starts before about age two is considered congenital.

P:  Are the terms "infantile" and "congenital" synonymous?    

Dr. Elliot Werner:  Not really.  "Congenital" specifically means present at birth.  "Infantile" could be acquired after birth.

P:  I recently learned that my 15-month-old daughter has a pale optic nerve, but she doesn't need glasses.  What is the significance of a pale optic nerve? 

Dr. Elliot Werner:  The optic nerve normally has a rosy pink color.  If it looks more white than pink, it is considered pale. The color of the nerve varies considerably from person to person.  Whether or not the nerve is abnormal can be difficult to determine, and usually requires more extensive testing than merely looking at it.

P:  What can a pale optical nerve lead to in the future?

Dr. Elliot Werner:  That depends upon the cause of the pale nerve.  Some conditions that produce atrophy of the nerve are progressive; others are not.

P:  The optical nerve appeared to look normal on the MRI (magnetic resonance imaging), but the ophthalmologist says it's pale.  What is the significance of his statements?  

Dr. Elliot Werner:  An optic nerve that is abnormally pale is atrophic, meaning that it has lost a significant number of its normal nerve fibers.  People with optic atrophy do not have normal visual function.  The MRI is not a good test to detect optic atrophy.  The nerve looking normal on MRI means there is no tumor pressing on the nerve to cause the atrophy.

P:  What tests determine pallor?  

Dr. Elliot Werner:  Pallor of the nerve is detected by looking at the optic nerve with the ophthalmoscope or taking a photograph.  Optic nerve pallor is a finding that points to some other underlying condition.  Whenever the doctor sees optic atrophy, he or she is then obligated to find out the cause. 

P:  Is MRI used to detect atrophy?  

Dr. Elliot Werner:  The MRI is used to detect some causes of optic atrophy, such as tumors and aneurysms. The MRI is not used to detect atrophy, but rather to explain it.

P:  How is a pale optic nerve related to agenesis of the corpus collusom and agenesis of the septum pellucidum?

Dr. Elliot Werner:  What you are describing is a rather rare congenital anomaly called DeMorsier's syndrome.  That is typically associated with optic atrophy and decreased vision, but is usually not progressive.

P:  My 15-month old daughter also has a lymphangioma close to the eye.  It will be removed in July.  Can that cause vision problems?

Dr. Elliot Werner:  It depends on what you mean by close to the eye.  If it is confined to the eyelid, usually not.

P:  It's on the bridge of her nose, close to her tear duct.

Dr. Elliot Werner:  That's unlikely to affect vision.

P:  She will be undergoing  a more extensive exam under anesthesia.  What will that exam be like?  Will it reveal if she will have seizures or has had mild seizures?

Dr. Elliot Werner:  The eye exam under anesthesia will be very similar to the exam an adult would have normally in the eye doctor's office.  I doubt that the doctor would be able to determine if she has seizures during an exam under anesthesia.  That would require a neurologic exam.

P:  How can it be determined that a baby needs glasses?  Can vision be a reason for a child not to walk,  or is that more a problem with balance?  

Dr. Elliot Werner:  You can dilate the pupil and use a procedure called retinoscopy to determine if glasses are needed in an infant.  This can usually be done without putting the baby to sleep.  Most blind children walk when they are supposed to.  The development of standing and walking seems to be independent of vision.  Blind babies, of course, are more likely to bump into things, so they may learn to be more careful in their walking.

P:  I have always been told I had congenital glaucoma, but symptoms of high intraocular pressure did not show up until after a retinal detachment and re-attachment.

Dr. Elliot Werner:  How old were you when you had the retinal detachment? 

P:  I was 14-years old.

P:  What are some of the special challenges in treating younger patients?

Dr. Elliot Werner:  In young children, compliance is a problem since it is difficult to give eyedrops daily.  Children are often more sensitive to the side effects of medications.  Older children and adolescents are often unwilling to admit they have a problem, and they resist treatment.

P:  Is compliance an issue with younger patients?  Or only after they become teens? 

Dr. Elliot Werner:  Both.   Young children who fight and struggle pose a problem for parents who are trying to get the drops in the eye.   Many children, unfortunately, do not have the kind of support and prenatal care at home to keep up with a difficult treatment regimen.  Teenagers are especially difficult and often rebel against having to deal with a chronic illness.

P:  Are you seeing more pediatric and congenital glaucoma these days?

Dr. Elliot Werner:  No.  The frequency seems to be fairly constant.

P:  Do all states in the U.S. require newborns to be checked for signs of glaucoma?

Dr. Elliot Werner:  I don't know, but I doubt it.  We depend on pediatricians and parents to detect something wrong.

P:  Are most primary glaucomas in younger patients open or closed angle?

Dr. Elliot Werner:  Almost all are open angle.  Closed-angle glaucoma in children is fairly uncommon, unless there is an associated condition such as retinopathy of prematurity or uveitis.

P:  When congenital glaucoma is diagnosed at birth, or even later, can something be done to prevent the onset of problems?

Dr. Elliot Werner:  You cannot prevent that condition.  With early diagnosis, the treatments are pretty good.  Most children can be managed so they do not go blind.

P:  Are there associated systemic diseases in a younger person that could be causing the glaucoma?

Dr. Elliot Werner:  A variety of congenital anomalies and acquired disease is associated with a higher incidence of glaucoma:  Down's syndrome, Marfan syndrome, and juvenile rheumatoid arthritis -- to name a few.

P:  Nail patella syndrome (NPS), too.

Dr. Elliot Werner:  Nail-patella syndrome is an extremely rare anomaly associated with glaucoma and a variety of other congenital deformities.  I have never seen a case and know very little about it.

P:  I have NPS, and my family is one of the families that showed the connection to nail-patella syndrome in Dr. Lichter's study. 

Dr. Elliot Werner:  You probably know more about it than I do.

P:  Eleven of eleven family members have glaucoma or are suspect.  Two have congenital glaucoma and one was diagnosed at age 16.

Dr. Elliot Werner:  That's not surprising.  I do know that congenital and juvenile glaucoma are common in NPS, but I have never seen a case. The hereditary syndromes tend to run in families.  Unless doctors have a family with the condition in their practice, they wouldn't normally encounter it.

P:  How important is it to find out the cause of a younger patient's glaucoma?  Will the cause of the glaucoma affect the treatment?

Dr. Elliot Werner:  It's very important to find the cause.  If there is a secondary glaucoma due to an underlying disease, that will drastically alter the treatment for many patients.

P:  Do you think the future looks brighter for younger glaucoma patients, with the promise of stem-cell research?

Dr. Elliot Werner:  That's hard to answer.  Regeneration of the retina and optic nerve is an important area of research.  The use of stem cells to regenerate central nervous system tissue is possible, but probably still far in the future.

P:  My five-month old baby was diagnosed with glaucoma at birth.  Her IOPs were 40 mm Hg in each eye.  Her doctor is patching the left eye to help it catch up.  When will we know how much damage was caused by the high pressures?  Are there any clues we can look for?  She seems to be seeing fairly well.  She swipes at toys and smiles.  On June 8 she had pupil surgery on her left eye to enlarge the pupil.  She could not see very well in that eye until after surgery.

Dr. Elliot Werner:  It is difficult to measure visual function in detail in a five-month-old baby.  The appearance of the nerve is the best indicator at that age.  Generally, you can begin to get a better assessment of visual function by age three or four years. 

P:  My baby's doctor said the optic nerve in her right eye is cupped 40%.  What does that mean? 

Dr. Elliot Werner:  That is not bad. It means that the optic cup occupies about 40% of the optic nerve, instead of the usual 10 to 20% in most babies. But 40% cupping is not bad and is consistent with almost completely normal vision.

P:  My little daughter had three surgeries, and Ahmed valves in both eyes at 9 days and 23 days.  She had a blocked tube and Ahmed revision on June 8. The pressure in her left eye was 28 mm Hg.  She had surgery to enlarge the pupil.  The results were dramatic.  She seems to see much better.  Can we do anything to help her with sight development?

Dr. Elliot Werner:  There is nothing specifically you can do.  As long as her disease is controlled and her retina and optic nerves are functioning reasonably well, vision should develop normally.

P:  What clues would indicate how well a baby is seeing?

Dr. Elliot Werner:  In young infants, we usually look for behavioral clues.  For example, does the child regard faces, fixate on small toys, appear to see new and interesting objects when they are introduced into the field of vision? 

P:  She does appear to look at faces, and her eyes are often riveted on nearby objects.  She swipes at toys and grabs and seems to be acting like any normal four or five-month old baby.  Does that sound about right?

Dr. Elliot Werner:  Sounds about right.  If she regards faces and small bright objects and reaches for them, that would be normal development for a five-month-old baby.

P:  She does not look right at our faces, but in the general direction.  She recognizes her father and me and smiles at us.  

Dr. Elliot Werner:  That is probably not unusual at five months.  Ask your pediatrician.

P:  I never knew a child could be born with glaucoma.  I have a lot to learn.  Our doctor said it is like running a marathon.  Now I know what he means.  I hope her pressures will become stable and we can concentrate on the seeing part.

Dr. Elliot Werner:  Having a child with congenital glaucoma is a burden and a commitment.  Our treatments for this condition, however, are pretty good.  A large majority of children do quite well and develop normally.

P:  Is there much networking among glaucoma specialists to keep up with what has worked well for the various secondary juvenile glaucomas?

Dr. Elliot Werner:  Yes.   The American Glaucoma Society has an Internet listserve where glaucoma specialists can post questions.  We ask our colleagues across the country for help.  The AGS (American Glaucoma Society) also meets annually. There is a lot of cross pollination going on in the glaucoma community.

P:  Are young glaucoma patients treated differently than older ones?  

Dr. Elliot Werner:  That depends upon the age.  In congenital glaucomas, the first-line treatment is surgical, rather than medical.  In older children, the treatment is similar to that of adults.

P:  What kind of surgery is performed for congenital glaucoma?

Dr. Elliot Werner:  The standard operation for congenital glaucoma is the goniotomy.  Some surgeons use a trabeculotomy. The results of the two procedures seem to be similar.

P:  I was told that even though many doctors perform goniotomies on children, only a small number of doctors use goniotomy for adults.  Does the procedure differ because of anatomy or disease process? 

Dr. Elliot Werner:  Most glaucoma specialists would say that goniotomies don't work in adults and should not be done.

P:  Thank you, Dr. Werner.  I am learning so much.  

[Editor's note:  The Glaucoma Foundation maintains an online forum for parents and relatives of young children with glaucoma, which "helps caregivers cope with the daily challenges of raising a child with glaucoma."  Information about "Young and Under Pressure for Parents," can be found at the following web address: http://www.glaucomafoundation.org/info.php?i=40.]

End of highlights for June 30, 2004.

On July 7, Dr. Wilson discussed "Infantile Glaucoma" in the Chat room. Click here for highlights of that meeting.

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