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    Updated classification gives order to corneal dystrophy

    Better diagnosis can help patients, physicians chart out best approach


    The document offers one-page descriptions of each dystrophy, how it is inherited, genetic information, the age of onset, signs and symptoms, how rapidly it progresses, and clinical photos.

    “There are levels of detail in the article that are certainly relevant to many doctors in terms of assisting them to make the diagnosis, helping inform the patient what the prognosis is, and the possibility of passing the disease on to their progeny,” Dr. Weiss said.

    Related: Creating corneal inlay pockets with femtosecond laser technology

    Some corneal dystrophies cause few symptoms. Others can lead to opacification, but corneal transplants can usually restore good vision in these patients, she noted.

    “One of my goals is for us to get to the next level that we have gotten to in many other diseases, where we can intervene to prevent disease progression and avoid visual loss,” she said.

    In the meantime, better diagnosis can help patients plan for the future, Dr. Weiss said.

    Recent: CXL continues to advance treatment of keratoconus, corneal ectasia

    “When we see a patient, they want to know what do they have and what can they expect as their life goes on,” she said. “Are they going to need surgery? Is their vision going to be impaired? What are the chances of them giving this to their progeny? And unless we really understand what we’re looking at, we can’t give the disease the correct name and most importantly we can’t really give the patient an accurate idea about what they may face in the future.”

    Dr. Weiss, an authority on Schnyder corneal dystrophy, gives the example of a young patient with this dystrophy who wanted to be a pilot. She was able to show the patient a chart indicating how the patient’s vision was likely to be affected at each age. The patient decided to become a pilot with the understanding that this career might not last past the age of 30 or 40 years.

    “Many times when I’m in a room with patients I’ll open the IC3D article and show them where they are,” Dr. Weiss said. “I find that my patients are always appreciative of information because knowledge is power.”

    In another case, she saw a Schnyder patient referred by another ophthalmologist who was considering laser treatment for corneal crystals. She pointed out that the treatment would not entirely restore the patient’s vision because of coexistent corneal haze underneath the crystals.

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    A correct diagnosis might help uncover systemic conditions as well. Since families with Schnyder often have elevated cholesterol levels whether or not the eye is affected, clinicians should advise these patients to have their systemic cholesterol levels checked, Dr. Weiss noted.

    “No one wants a disease, but the unknown is often more frightening than the known,” she said.


    Jayne S. Weiss, MD

    E: [email protected] 

    This article was adapted from Dr. Weiss’ delivery of the Richard L. Lindstrom, MD, Lecture during the 2016 meeting of the American Society of Cataract and Refractive Surgery. She did not indicate any proprietary interest in the subject matter.

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