Exfoliation Syndrome
Chat Highlights
October 5, 2011

On Wednesday, October 5, 2011, Dr. Michael Pro, a glaucoma specialist at Wills, and the glaucoma chat group discussed "Exfoliation Syndrome".

Moderator: Tonight's topic is “Exfoliation Syndrome”. Dr. Pro, what is it?

Dr. Pro: Exfoliation syndrome (XFS) is an age-related, systemic, elastic microfibrillopathy affecting both intraocular and extraocular tissues. The clinical features include: white flake-like material on the anterior lens surface, the pupillary border, trabecular meshwork, zonula, ciliary body, and other anterior segment structures. XFS is a major risk factor for the development of glaucoma.

P: What is pseudoexfoliating glaucoma?

Dr. Pro: That is glaucoma due to the syndrome I described above. Basically, you can think of it as leading to deficiencies in aqueous outflow (drain problem!). You see, in "typical" primary open angle glaucoma, we really don't know why the outflow is deficient. The drain looks just fine! In XFS, we assume that the abnormal anterior segment material and excess pigment is somehow toxic to the aqueous drainage system.

P: Is there a difference between pseudoexfoliative syndrome and exfoliation syndrome? Is there a difference between pseudoexfoliative glaucoma and exfoliation glaucoma?

Dr. Pro: No, historically there was a condition called exfoliation syndrome seen in glassblowers who were exposed to extreme heat. The lens capsule looked peeled like an onion. Later a similar appearance was found in other individuals and was termed "pseudo" exfoliation syndrome. Today, we use both term as the original condition is really no longer found.

P: Are there any other names this condition could be called?

Dr. Pro: No there are not.

P: Since exfoliation glaucoma and traumatic glaucoma both affect only one eye, is it easy to differentiate the two types of glaucoma?

Dr. Pro: Oh yes. Traumatic glaucoma has a history of significant trauma (usually much more than a mundane bump or bruise). Traumatic glaucoma often has notable iris findings (iris root tears, etc). Pseudoexfoliation glaucoma (XFG) has the anterior segment findings that I noted above.

P: Where does the amyloid material found in the eye that flakes like dandruff come from?

Dr. Pro: It is thought to be abnormal cellular basement membrane material. Incidentally, in individuals with XFS, the amyloid material can be found in other parts of the body. The significance of this is not clear.

P: Can this material be seen with the naked eye?

Dr. Pro: No, only at the slit lamp.

P: In the last chat about this form of glaucoma, you mentioned a study showing this is a systemic disease. Does the condition first appear in the eye or in the vascular system?

Dr. Pro: Great question, every review I have seen is in patients where the condition has first been found in the eye.

P: Does the medication used to treat the vascular system disease affect the condition in the eye?

Dr. Pro: There is no systemic treatment for XFS. Let me add a few lines about XFG. Exfoliation glaucoma (XFG) is characterized by rapid progression, high resistance to medical therapy, and poor prognosis. XFS can not only lead to severe chronic open-angle glaucoma but also to acceleration of cataract formation, lens subluxation, angle-closure glaucoma, and severe complications at the time of cataract extraction, such as zonular dialysis, capsular rupture, and vitreous loss.

P: Do the eye drops used to initially treat the eye condition affect the vascular system?

Dr. Pro: No. Let me be clear that although the material may be found in the vascular system, there is conflicting evidence that XFS leads to increased cardiac disease.

P: Is there less of the amyloid material found in the bodies of the population that are not suffering from vascular disease or exfoliating syndrome but are in their sixties and seventies?

Dr. Pro: I'm sorry, I do not know the answer to that. My understanding of the condition relates to its effects on the eye, where it is a major cause of glaucoma, especially in certain populations.

P: What role do genetics play in this condition?

Dr. Pro: According to studies worldwide, there is evidence showing that genetic factors may play an important role in the pathogenesis of XFS. The gene is called LOXL1. Maybe someday we will test individuals at risk for XFS/XFG for this gene.

P: I've developed a number of conditions (said to be unconnected) that have made me (and my family doctor) suspicious that I may have an amyloidosis issue (free light chain assay slightly high for kappa, kappa/labda ratio slightly high, and fat pad biopsy negative). Reading about XFG, I wondered whether this may also be connected to amyloidosis, and how do you ascertain that it's not. Also, if it's due to a vascular issue, how due you know that the optic nerve damage isn't due to a vascular deficiency rather than just high IOP.

Dr. Pro: XFS is not related to the condition that you describe. The material is called amyloid, but that is more of a catch-all term for this acellular fibrillar build-up seen in XFS. The reason that we do not think vascular disorders are the main issue in XFG, is that in these patients, the IOP is typically higher than average and very labile.

In fact a major glaucoma trial helped to clarify this. Please allow me to describe this trial. The Early Manifest Glaucoma Trial (EMGT) is a randomized clinical trial that demonstrated the effectiveness of immediate therapy to lower the IOP, versus no initial treatment, on progression of early, newly detected OAG (including primary, "normal-tension," and exfoliation glaucoma types). The EMGT results demonstrated that treated patients had half the risk of progression compared with controls. In addition, each millimeter of mercury higher of mean IOP during follow-up was associated with a 13% increased risk of progression. But, in patients with early glaucoma, IOP remained stable without treatment during a six year period, regardless of baseline IOP, except for patients with exfoliation glaucoma, where IOP increased by almost 1 mm Hg annually.

P: I hope this is not a silly question. I have used eye makeup all my life. If the iris rubs against the lens and the particles flake off, I would think that the nightly rubbing that I do with my eye makeup remover might not be a good idea.

Dr. Pro: Wow, that is a great theory. I think you may have to really rub your eye to actually cause the iris pigment to flake! There is an ocular disorder that may be related to chronic eye rubbing and that is keratoconus, where the cornea becomes thin and irregularly shaped.

P: Are they any restrictions in activity or things these folks should avoid?

Dr. Pro: No.

P: SLT (laser) makes burns holes in the trabecular meshwork. If this condition clogs the meshwork with the amyloid material, why doesn’t the laser always work at lowering eye pressure by renewing the meshwork?

Dr. Pro: That is not how the SLT works. In fact, there is no discernable change in the structure of the trabecular meshwork (TM) after an SLT. It is thought that the SLT causes a mild inflammatory reaction in the SLT which leads to an improvement in the outflow facility through the TM. In fact, SLT may not work as well on average in XFG, but that may be due to irreversible changes in the TM.

P: Do exfoliation glaucoma patients develop blepheritis more often than the total glaucoma population? If so, why?

Dr. Pro: Your question is really great. I have not seen an association of more blepharitis and XFG and I do not know that an association has been described in the medical literature.

P: Of those who have XFS, what percentage develop XFG?

Dr. Pro: Awesome question. It can be as high as 60 percent, 15 years after initial diagnosis with XFS.

Moderator: That's it folks! Thank you Dr Pro.

Dr. Pro: Great work tonight everyone! Good night!

Click here for the most recent glaucoma chat highlights and links to the chat archives.

Click here for upcoming glaucoma chat events.