Which are the symptoms of Axenfeld-Rieger Syndrome?

The iris atrophy and glaucoma are at the top of the list, followed by the dental deformities.

In my 66 year journey with ARS, the symptom that is the most challenging to live with is iris atrophy. About 10 years ago we began treating it by using tropicamide to dilate my eye every day. This significantly reduced the symptoms for several years. However, beginning a couple of years ago, the symptoms have gradually gotten worse in spite of increasing the strength and frequency of the medication. I would gladly give up the growing darkness in my life and the “sheer curtain” between the world and me.

Another aspect of ARS that is difficult for me is the genetic dominance of the trait. Although we had been dealing with abdominal, oral/dental and visual symptoms since my birth; I was 26 years old when, during a routine eye exam (-13 diopters corrective lenses) my glaucoma (IOPs mid-50s, visual fields reduced to lower half) was first diagnosed and I was introduced to Riegers. (Axenfeld came later). I was asked if anyone else in my family had glaucoma. No. I was asked if I had children. Yes. It was like being punched in the stomach. My son was three years old at the time, and yes, I passed ARS to him. Forty years later it continues to be a painful burden to bear.

Early onset narrow angle glaucoma, untreatable with conventional medicines and often requires surgery. Thickened corneas prone to edema and epithelial cells non-receptive to transplants (99% success rate normally)