Axenfeld - Riegers syndrome- glaucoma

  Our son age 7 was dx with Axenfeld Reigers syndrome. It is very rare- it is also called anterior segment dysgenesis.  He is at a high risk for closed angle glaucoma and we have his pressures checked regularly.  I am trying to get some info on how the Closed angle glaucoma would come on.  The docs dont know much about this- actually no one seems to as it only occurs in 1 in every 200,000 people.  Do the symptoms of the closed angle glaucoma come on quickly over days or hours ect?  Most of the info   I have found is on open angle glaucoma.  ANy info on this would be appreciated!  Thanks!  

[HFHS M.D.-NI]

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Ruth,
Axenfeld - Reigers syndrome is a continuum of anterior segment developmental abnormalities which can predispose to glaucoma.  This occurs in about 50% of patients with the disease.  Some of the drainage angle is closed.  As long as enough of the angle is open and properly functioning, the eye pressure should stay normal.  As the disease progresses, the open part of the angle can close and become dysfunctional.  This is not like acute angle closure glaucoma where the pressure can be normal at one time and become dangerously elevated within 30 minutes.  The treatment for acute angle closure glaucoma involves making a small hole in the iris.  That will not work in Axenfeld - Rieger as the cause for angle closure is different.
It seems like you are doing the right things.  Your son should be regularly examined for signs of glaucoma.  This would include checking the eye pressure, the optic nerve appearance and the drainage angle.  Once he is able to cooperate, he should also have serial visual field examinations.
It is important to maintain close follow-up as this condition usually affects both eyes.  There can also be a hereditary component so any other children should also be screened.
This information is provided for educational purposes only.  Please see your physician for diagnostic and treatment options.

Sincerely,
HFHS MD - NI
*keywords: Axenfeld-Rieger syndrome, Anterior segment dysgenesis, angle closure, glaucoma